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STUDY GUIDE
2.2 EDITION
Senior Editor
Norman H. Ertel, MD
Associate Editors
James M. Horowitz, MD
Miguel A. Paniagua, MD, FACP
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al Testing and Assessment Systems, Inc. which controls the content and owns all copyrights
in the materials.
The developments in medicine are always changing, from clinical experiences, new research, and
changes in treatment and drug therapy. The Internal Medicine In-Review team use reasonable efforts
to include information that is complete and within accepted standards at the time of publication.
However, the faculty, authors, publisher, nor any other party who has been involved in the preparation
of Internal Medicine In-Review make no representations, warranties, or assurances as to the accuracy,
currency, or completeness of the information provided. Users of Internal Medicine In-Review are
encouraged to conrm the information contained with other sources. The publishers of this guide
shall not be liable for any damages or injury resulting from your access to this guide, or from your
reliance on any information provided in this guide. This Study System is intended for U.S. physicians
only and is not in any means intended for use by the general public.
ISBN 978-0-9858025-1-6
2014 Published by Educational Testing & Assessment Systems, a product of SanovaWorks
Edited by Norman H. Ertel, MD
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Challenge Yourself with These Case Study Questions
2. An 80 year-old woman presents with dysphagia, epigastric pain, and frequent belching. Her symptoms are particularly
bothersome in the morning after eating breakfast. Her esophagram is shown. What is the most likely manometric finding?
3. A 40-year-old competitive athlete has noticed intermittent irregular pulse while running but no other associated symptoms.
On examination he has normal cardiopulmonary exam and BP is recorded as 122/78. EKG and echo are normal. He was set
up for a Holter monitoring and a strip from Holter (while asleep) shows Wenckebach 2 heart block. Which of the following would
be the most appropriate next step in management?
a. Angiography
b. Pacemaker For More Free MCQs
c. Thallium stress test & to Find Out How You Did, Visit
d. Exercise stress test
InReviewIM.com/studyguide
e. Reassure
Users Guide
Created for first-year residents to use throughout the duration of their residency training, the Internal Medicine In-
Review Study Guide is formatted for quick and streamlined studying. When reviewing the guide, it is important to
keep in mind the following:
Need a refresher? Internal Medicine In-Review is not meant to be a reference guide with an intimidating sizethe quick,
need-to-know bulleted take-away points are designed for high-yield studying. This guide is meant for the residents that lack
time in their schedules to read in-depth information.
This Study Guide is not a stand-alone resource; it was created as part of a multi-component study system. It is crucial that
you supplement your reading by challenging yourself with Board-type questions and interactive study tools, available on the
companion app and website: InReviewIM.com.
You will notice redundancy between chapters. Repetition has been purposely integrated to further your learning.
Compiled of 17 chapters, the Study Guide covers the American Board of Internal Medicine (ABIM) certification exam
blueprint. You will also note a pain management chapter, which is not expressly identified as a content area covered on the
ABIM certification exam; however, pain management can be found throughout many of the medical-content categories,
including the 3% of the exam referred to as Miscellaneous.
Each chapter is followed by a series of Notes
pages that should be used for your own
annotations, study tips, and mnemonics. You
may also make use of the ample margin space
Dont forget to check out the Online
throughout the chapters. Study System for hundreds of multiple-choice
To help us make this a dynamic and ever- questions, detailed answer explanations,
improving resource, please share with us your printable flashcards, and a timed practice exam
thoughts and your annotations. You may email
us at info@etasonline.com. simulation at InReviewIM.com.
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ERR A T A
The following errata is a list of errors and their corrections for the enclosed Internal Medicine In-Review Study Guide. Please visit the Online Study System for the fully
updated 2nd Edition, which includes optimizations and improvements to the Study Guide not listed below. You can download a PDF of the 2nd Edition at InReviewIM.com.
Module 1
Page # Section Correction
4 Urinary Incontinence Under Anticholinergics, include oxybutynin, tolterodine, darifenacin, solifenacin, trospium, and fesoterodine
18 OTC Pain Medications Subsection of NSAIDs, add New generation NSIADs such as Cyclooxygenase-2 ( Cox-2 ) inhibitors such as celecoxib can
provide better safety profile compared to traditional NSAIDs
21 Complementary and Alterna- Under Evaluation of patients for abuse potential add: ioids titration to long acting meds. Under Complementary and
tive Medicine (CAM) Alternative Medicine (CAM) add Physical Therapy, Chiropractor, aqua therapy, bio feedback
26 Headache and Facial Pain Under Surgical therapy is reserved to TN that is refractory to medical therapy change as follows: Nerve blocks, Microvas-
cular decompressin, Nerve Ablation, Chemical, Radiofrequency ablation, Gama Knife Radiosurgery
28 Treatment of Low Back Pain Under Subacute and Chronic Low Back PainAdd line between exercise and surgery lines as follow:- Interventional pain
procedures such as nerve blocks, Epidural injections, Sacro-Iliac joint injections, Facet nerve blocks, Radio frequency abla-
tion etc.
28 Neck Pain Add interventional approaches in multifaceted therapy as follow: Interventional pain procedures such as nerve blocks,
Epidural injections, Facet nerve blocks, Radio frequency ablation etc
31 Hip Pain Under Trochanteric Bursitis add: Bursa injection for Trochanteric bursitis not responding to conservative treatment
33 Knee Pain Subsection of Remember: Change Acute knee pain secondary to trauma can be a result of injuries to Acute knee pain
secondary to trauma can be a result of sports injuries
35 Fibromyalgia Syndrome Update diagnostic criteria of Fibromyalgia. As follow: Remove elicits painful response at 10 or more of the 18 tender points:
Occiput area, upper back, neck, upper chest, elbows hips, and knees update with 2010 PRELIMINARY DIAGNOSTIC CRI-
TERIA (EXCERPT) AS PER AMERICAN COLLEGE OF RHEUMATOLOGY (ACR) includes A patient satisfies diagnostic criteria for
fibromyalgia if the following 3 conditions are met: Widespread pain index (WPI) 7 and symptom severity (SS) scale score
5 or WPI 3 - 6 and SS scale score 9. Symptoms have been present at a similar level for at least 3 months. The patient
does not have a disorder that would otherwise explain the pain. Add: Muscle relaxant in medications list
44 History: Table 3.1 Under Conductive hearing loss, include foreign bodies as a cause
Under Sensory hearing loss, include furosemide as a cause
51 Sinusitis: Treatment After silver nitrate can be applied topically to cauterize the bleeding site of a patch can be applied, include before apply-
ing silver nitrate, numb the nose bleed with lidocaine.
79 Intrauterine Devices In the first bullet point, include (LNG) after levonorgestrel
Module 2
Page # Section Correction
3 Acute Ischemic Stroke Under Evaluation and Treatment new guidelines, change If <3 hours to If >3 to 4 1/2 hrs since..
6 Demyelinating Diseases Add Guillen Barre (GBS) Syndrome
(Other)
10 Movement Disorders (Other) Under Huntington disease change between 20 to 40 years of age to age less than 40 years of age
12 Normal Pressure Hydroceph- Add last bullet: Clinical Pearl 3 Ws as a pneumonic - Wet, Wobbly, Weird
alus (NPH) See it everywhere, will help people to remember
116 Hematology Chapter Please see additional sections to the hematology chapter by viewing the Online Study Guide at IMInReview.com
Module 3
Page # Section Correction
30 Meningitis Replace first bullet with, The classic triad is fevers, altered mental status, and stiff neck.Include the word often at the
beginning of the sixth bullet so that it reads, Often, normal CSF glucose essentially rules out acute bacterial meningitis
(ABM)
32 Encephalitis: Table 14.2 Bullet point that says, PMN predominance early, later shifting to lymphocytes under HSV-1: Historical/Laboratory Clues
should be replaced with, PMN predominance late (after 1 week after onset) for most people, with lymphocytic predomi-
nance typically at the time of presentation
33 Neurosyphilis IV penicillin G is preferred therapy should be changed to, IV penicilli G is the required therapy. Those that cannot tolerate
it should be desensitized. All other regiments are suboptimal and with high failure rates
36 Community Acquired Pneu- Oral anaerobes should not be italicized
monia (CAP): Table 14.4 Under treatment for Oral anaerobes, current options should be replaced with the following, clindamycin or a beta-lactam
+ clindamycin/metronidazole or ampicillin/sulbactam or moxifloxacin
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E R R A T A
Module 3
Page # Section Correction
44 Acute Infectious Diarrheas: E. Replace erythromycin with azithromycin in the following sentence, Treat Campylobacter with erythromycin or a
Coli quinolone.
45 Urinary Tract Infections Remove antibiotic therapy is option from the 6th bullet point
48 Epididymitis The last bullet, which starts with Blastomyces should be treated has a misspelling. Itraconozole should be
replaced with itraconazole
90 Barrett Esophagus Under Screening and Diagnosis add after esophageal adenocarcinoma Risk factors : age> 50 years, white, male,
obesity, chronic GERD
90 Barrett Esophagus Under Treatment Add: UPDATED GUIDELINE: Endoscopic therapy with RFA, endoscopic mucosal resection (EMR) and
photodynamic therapy (PDT) should be attempted in high-grade Barretts before surgery [1]
91 Esophageal Carcinoma Please see new additions to the Disorders of the Esophagus chapter by viewing the Online Study Guide at IMInReview.
com
92 Esophageal Motility Disorders Under Diffuse Esophageal Spasm and Nutcracker Esophagus add: Chest pain, dysphagia, globus sensation, regur-
gitation
add after second bullet Barium swallow shows cock-screw esophagus After channel blockers ADD: proton pump
inhibitors, botulinum toxins, and antidepressants
93 Infectious, Pill-induced, and Delete this statement: Candida albicans is the most common organism causing esophagitis in immunocompetent
Eosinophilic Esophagitis patients
Replace with: The most common cause of esophagitis in immunocompetent patients is GERD, about 20-30% of cases.
ADD: Fungal (Candida albicians) and Viral (HSV and CMV) esophagitis are seen in immune-compromised patients
93 Disorders Of The Stomach And Add Classification: Gastric ulcer (worsens with eating, in the upper Epigastric region) Duodenal ulcer (improves with
Duodenum eating, but worsens 3 hours after eating)
94 Helicobacter Pylori Infection After Urea breath testing ADD: Diagnosed by the Urea breath test : patient drinks C-13 0r C-14 labelled urea which
the bacterium metabolizes, producing labelled carbon dioxide that can be detected in the breath
95 Gastric Polyps, Subepithelial After: Weight loss, Add: bleeding, dysphagia, and upper abdominal pain
Lesions, and Adenocarcinoma Add bullet: Diagnosis: gastroscopic exam using a fiberoptic camera to visualize the gastric tissue is standard diagnostic
method; followed by biopsy. Add bullet: Prognosis : less than 10% 5-year survival rate.
96 Disorders Of The Intestines Please see additional sections to the Disorders Of The Intestines chapter by viewing the Online Study Guide at
IMInReview.com
102 Chronic Pancreatitis After ANA titer ADD: for a duration of 4-6 weeks, and repeated imaging used to monitor for improvement -Recent
update guidelines: Patients with IgG4 autoimmune pancreatitis have a higher risk of relapse if not maintained on an
immunosuppressant, and should be maintained on azathioprine
102 Chronic Pancreatitis Under Diagnosis: DELETE: Contrast-enhanced helical computed tomography (CT) scanning is the preferred method to
diagnose and stage pancreatic cancer ADD: Transabdominal Ultrasound is the first line because of its higher sensitiv-
ity (>90%) for all pancreatic tumors; in contrast, triple-phase helical multi-detector row CT abdomen is 100% sensitive
for tumors >2cm, but 77% sensitive for tumors <2cm.
105 Disorders Of The Liver, Please see additional sections to the Disorders Of The Liver, Gallbladder, And Bile Ducts chapter by viewing the Online
Gallbladder, And Bile Ducts Study Guide at IMInReview.com
119 Medical Oncology Please see additional content added to Medical Oncology chapter by viewing the Online Study Guide at IMInReview.
com
Any questions about the content of Internal Medicine In-Review should be directed to Educational Testing and Assessment Systems, Inc.
which controls the content and owns all copyrights in the materials.
The developments in medicine are always changing, from clinical experiences, new research, and changes in treatment and drug therapy.
The Internal Medicine In-Review team use reasonable efforts to include information that is complete and within accepted standards at the
time of publication. However, the faculty, authors, publisher, nor any other party who has been involved in the preparation of Internal Medicine
In-Review make no representations, warranties, or assurances as to the accuracy, currency, or completeness of the information provided.
Users of Internal Medicine In-Review are encouraged to confirm the information contained with other sources. The publishers of this guide
shall not be liable for any damages or injury resulting from your access to this guide, or from your reliance on any information provided in this
guide. This Study System is intended for U.S. physicians only and is not in any means intended for use by the general public.
2017 Educational Testing and Assessment Systems (ETAS). All Rights Reserved. This document contains proprietary information, images, and marks of ETAS.
No reproduction or use of any portion of the contents of these materials may be made without the express written consent of ETAS. Licensed to gee.icloud@icloud.com.
INTERNAL MEDICINE IN-REVIEW
STUDY GUIDE
2.2 EDITION
MODULE 1
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INTERNAL MEDICINE IN-REVIEW
STUDY GUIDE
2.2 EDITION
MODULE 2
FACULTYv
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INTERNAL MEDICINE IN-REVIEW
STUDY GUIDE
2.2 EDITION
MODULE 3
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1 General Internal Medicine
Fred Buckhold, MD
C o n t e n t s
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1.1 ROUTINE CARE OF THE HEALTHY PATIENT
Screening
Assessing patients for risk or presence of asymptomatic disease and identifying lifestyle
choices that have health consequences.
Often based on prevalence of diseases
The United States Preventative Services Task Force (USPSTF) has recommendations on
which screening measures are effective
Screening During History and Physical Exam
Measure height and weight (for BMI/obesity)
Measure blood pressure (for hypertension)
Assess for tobacco, alcohol, and drug use
Detailed sexual history, including risk factors for STDs
Screening Tests
Papanicolaou test every 3 years starting at age 21, stopping age 65
Chlamydia for sexually active women under age of 25
Routine HIV screening for ages 13 to 64
Biennial mammograms for women age 50 to 74
Cholesterol screening (Total and HDL) every 5 years in men >35 years and women >45
years
Fasting glucose if sustained blood pressure >135/80 mmHg
Colonoscopy every 10 years above age of 50 until age 74; may also have occult blood
testing yearly or flexible sigmoidoscopy every 5 years
Abdominal aortic aneurysm for male adults between 65 to 75, if history of smoking present
Osteoporosis (via BMD) in women >65, or 60 to 64 if body weight below 70 kg
Family History
Taking a Family History
Screen for diseases that family members have
Inquire specifically about early onset cardiovascular disease and 1st and 2nd degree
relatives with cancer (and what type/age of onset)
Genetic Testing
More useful for high risk populations, or if suspected
Testing should aid in diagnosis and/or management
BRCA or FAP mutation may lead to aggressive screening and interventions
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If dependencereferral to treatment
Illicit drugs: Look for behavior changes, legal troubles, and medical sequelae
Referral or brief counseling
Sexual Behavior
History oriented to high risk practices (men who have sex with men, multiple partners,
contact with sex workers)
Counsel of safe sex practices (use of barrier protection vs. birth control for prevention of
STDs)
Offer screening for STDs as appropriate
Domestic Violence
Screen if suspected
Intervene if possible; assessing for safety, clear documentation of injuries, and providing
resources
Urinary Incontinence
Stress: Loss of sphincter tone, worse with cough
Pelvic floor exercises
Estrogen cream
Surgery
Urge: Spastic/overactive detrusor muscle
Timed voiding
Anticholinergics: oxybutynin, tolterodine, darifenacin, solifenacin, trospium, and
fesoterodine
In men: Consider overflow incontinence from bladder retention from BPH
Pressure Ulcers
Prevent them at all costs, aimed at reducing friction, shear stress, and skin moisture.
May need debridement and antibiotics for stage III-IV ulcers
Cardiac Evaluation
ACC/AHA algorithm
1. Emergent surgery to OR
2. Active cardiac condition (ACS, ventricular arrhythmia, decompensated heart failure)
treat condition
3. Low risk surgery to OR
4. Assess functional capacity (can walk up a flight of stairs or hill go to OR)
5. If unable to assess or perform at functional capacity, assess risk factors; if 3 or more
consider testing if it will change management
Risk factors
High risk surgery
Creatinine >2.0
Ischemic heart disease
History of stroke
Diabetes requiring insulin
LV systolic dysfunction with EF <35%
If patient has coronary revascularization prior to surgery (either due to pre-operative
assessment or not), needs to be on anti-platelet agents for 4 to 6 weeks for bare metal
stent, 12 months for drug eluting stent
Both aspirin and clopidogrel should be stopped about 5 days before planned surgery
Maintain b-blocker dose if already takingno need to add before surgery
Consider stopping lisinopril
Pulmonary Evaluation
Risk factors associated with pulmonary complications
Type of surgery (emergent vs. non-emergent) and surgery duration
Location (intrathoracic and upper abdominal highest risk)
Use of general anesthesia, long acting neuromuscular blockers, and NG tubes
Only measure to reduce morbidity/mortality is incentive spirometry postoperatively
Only do diagnostic spirometry pre-operatively if having lung resection
Chest radiograph not needed
VTE Prophylaxis
Should be applied to most surgical patients (mechanical and pharmacologic)
Usually LMWH or heparin is sufficient
Higher risk surgeries (hip or knee arthroplasty, hip fracture, trauma, spinal injury):
Therapeutic warfarin for INR 2-3 or fondaparinux
Glycemic Control
Diabetics should be monitored closely peri-operatively
Data unclear on optimal regimen, likely benefit if blood sugar remains less than 180 mg/dL
Continue insulin regimen (basal, no bolus) for type 1 diabetics
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Halve insulin for type 2 diabetics the day of surgery
Metformin and other agents should be stopped 1 to 3 days prior to operation
Other Considerations
Surgery in a hyperthyroid patient may induce thyroid storm
May use steroids to block this effect
Stress steroids may be required for steroid-dependent patients
Dialysis patients should ideally be dialyzed the day prior to surgery
Delirium commonly occurs in patients at risk
Symptom Management
Pain: Aspirin, acetaminophen, or NSAIDs initially, at labeled dosing. For severe pain, opioids
are reasonable
Can use combination regimen of long acting and short acting opioids
Oral is preferred, avoid meperidine and opioid agonists/antagonists
Liquid morphine may be helpful for those with difficulty swallowing
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Monitor for side effects
Surgery may be useful for visceral pain
Appropriate agents for neuropathic pain include TCAs, dual acting anti-depressants,
tramadol, gabapentin, pregabalin
Dyspnea: Treat the symptom
Use short acting opioid if on chronic therapy for symptom relief
Morphine also helpful
Do not use benzodiazepines
Oxygen for hypoxemia
Nutrition: Appetite may wane, but important to consider social element of eating
If not near death, stimulants are appropriate - megestrol and medroxyprogesterone
Unclear benefit of enteral/parental feeding
Depression: Difficult to diagnose
Patients noting low mood and low interest are more likely to be depressed
Treat with SSRIs or TCAs
Altered mental status
Consider medication, especially opioids
Consider prognosis before full evaluation
Haloperidol may be used for symptoms, can also use benzodiazepine with monitoring
Caregiver stressors and bereavement
Must be closely monitored
If symptoms of bereavement continue for 6 months, high risk for complicated grief and
depression may need treatment medically or with therapy
Dizziness
Helpful exam maneuvers
Orthostatics for lightheadedness
Dix-Hallpike for positional vertigo
Neurologic and ear assessment for vertigo
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Vestibular and Peripheral Nerve Causes of Dizziness
Usually related to vertigo
BPPV use Epley maneuver to improve
Vestibular neuronitis likely after viral infection; symptoms not self limiting
May respond to steroids
Meniere vertigo with hearing loss and tinnitus
Thiazide diuretics to treat
Central Nervous System Causes of Dizziness
Rarely tumor
Vascular disease of posterior cerebral arteries; consider when other vascular risk factors
present
Migraine headaches
MRI may be helpful if symptoms or exam suggest
Disequilibrium Gait Unsteadiness
Multifactorial peripheral neuropathy, poor eyesight, medications
Safety features added to homes, assistive devices, and reducing polypharmacy helpful
Non-specific Dizziness
May be related to other systemic problems (hypoglycemia, hyperventilation)
Associated with psychiatric illness
Insomnia
Often related to co-morbidities
Take a detailed history involving time going to sleep, duration, wakefulness upon waking
Non-drug therapies (sleep restriction, cognitive therapy, sleep journal) likely more effective
than drug therapies
Can use medication, preferably not benzodiazepines
Syncope
Transient loss of consciousness due to lack of cerebral blood flow. Pre-syncope is the sensa-
tion of almost losing consciousness. Pathophysiology typically the same for both.
Patients with high index of suspicion for heat disease (based on previous history or ECG
changes) should be admitted for evaluation
Evaluation should be based on suspected underlying condition
Chest Pain
Priority in differential diagnosis depends on situation
Majority of CP in ambulatory clinic musculoskeletal
In emergency settings acute coronary syndrome
Differential is broad; clues to diagnosis include:
Ischemic changes to heart: Substernal pain or tightness with radiation; ECG and
biochemical changes
Reproducible, sharp pain, negative ECG, or positional-related makes ischemia
unlikely
Aortic dissection abrupt onset, tearing chest pain, pulse differential, wide
mediastinum on chest X-ray if considering, order MRI, angiography, or chest CT (not
echo)
Pulmonary embolism pleuritic pain, sudden onset, dyspnea, tachycardia, and risk
factors; CT angio is reasonable if intermediate or high suspicion
Pneumonia fever, productive cough, infiltrate on chest X-ray
Gastroesophageal reflux often mimics ischemic heart disease, always consider cardiac
disease. Trial of proton pump inhibitor if cardiac disease ruled out
Musculoskeletal reproducible tenderness, otherwise normal exam
Panic attack or anxiety
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Hyperlipidemia
Screening males >35, females >45
Components of cholesterol profile
LDL main substrate for atherogenic disease. Usually elevated due to dietary/lifestyle
or genetic causes, also consider hypothyroidism, nephrotic syndrome, obstructive liver
disease, and certain medications
Primary target of cholesterol lowering therapy
Triglycerides not reliable predictor of cardiovascular disease; if high, may affect
estimation of LDL (LDL not usually measured directly)
Must treat if above 500 mg/dL to decrease risk of pancreatitis
HDL higher levels correlate with decreased incidence of cardiovascular disease; no
evidence that raising improves CAD risk
Treatment
Lifestyle modifications 1st choice restriction of fat, more plant stenosis, exercise
Statins are mainstay of secondary prevention (for those who have CAD), and should be
considered for primary prevention if risk is high enough
Must be vigilant for effects of myopathy and myalgia, elevated liver enzymes, and
rhabdomyolysis
Other drugs fibrates (mainly gemfibrozil), ezetimibe, and niacin may be used to
further reduce LDL or improve HDL, but benefit on atherogenic disease is uncertain
Goals to treat with medication
Use Framingham Risk Score (FRS) to assess 10 year cardiovascular risk
Risk factors for CVD
Cigarette smoking
Hypertension
Age (men 45, women 55)
Low HDL (<40 mg/dl)
Family history male relative with CAD <55, female <65
If CVD present, treat LDL 100 mg/dL
If FRS 10 to 20%, 2 risk factors, treat if LDL 130 mg/dL
If FRS <10%, 2 risk factors, treat LDL 160 mg/dL
If FRS <10%, 0 to 1 risk factors, treat LDL 190 mg/dL
If LDL is lower than above numbers, but elevated, start lifestyle changes
Obesity
Defined as BMI >30
Obesity confers higher risk of chronic disease
Evaluation should probe for causes, including:
Medication induced
Endocrine hypothyroid, excess cortisol, polycystic ovarian syndrome
Other cardiovascular comorbidities (hypertension, diabetes, dyslipidemia)
Treatment
Diet and exercise
Behavior modification
Orlistat and sibutramine can be used as supplement to above
Sibutramine should not be used in hypertensive patients
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REFERENCES & SUGGESTED READINGS
1. U.S. Preventive Services Task Force. Screening for breast cancer: U.S. Preventive Services Task
Force Recommendation Statement. Ann Intern Med2009;151:716-726.
2. U.S. Preventive Services Task Force.Screening for Colorectal Cancer: U.S. Preventive Services Task
Force Recommendation Statement. AHRQ Publication 08-05124-EF-3, October 2008. http://www.
uspreventiveservicestaskforce.org/uspstf08/colocancer/colors.htm
3. Screening for Cervical Cancer, Topic Page. April 2012. U.S. Preventive Services Task Force. http://
www.uspreventiveservicestaskforce.org/uspstf/uspscerv.htm
4. Centers for Disease Control and Prevention. Recommended adult immunization scheduleUnited
States, 2012. MMWR 2012;61(4).
5. Fleisher LA, Beckman JA, Brown KA, Calkins H, Chaikof E, Fleischmann KE, Freeman WK, Froehlich
JB, Kasper EK, Kersten JR, Riegel B, Robb JF. ACC/AHA 2007 guidelines on perioperative car-
diovascular evaluation and care for noncardiac surgery: a report of the American College of
Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee
to Revise the 2002 Guidelines on Perioperative Cardiovascular Evaluation for Noncardiac
Surgery).Circulation. 2007;116:e418e499.
6. McGlynn EA, et al. The quality of health care delivered to adults in the United States. N Engl J Med.
348:2635.
7. Snyder L, Leffler C; Ethics and Human Rights Committee; American College of Physicians. Ethics
Manual: fifth edition. Ann Intern Med. 2005;142(7):560-582.
8. Qaseem A, Snow V, Shekelle P, et al; Clinical Efficacy Assessment Subcommittee of the American
College of Physicians. Evidence-Based Interventions to Improve the Palliative Care of Pain, Dyspnea,
and Depression at the End of Life: A Clinical Practice Guideline from the American College of
Physicians. Ann Intern Med. 2008;148(2):141-146.
9. Keith M. Swetz, Arif H. Kamal, Deborah Cotton, Darren Taichman, Sankey Williams; Palliative Care.
Annals of Internal Medicine. 2012 Feb;156(3):ITC2-1.
10. Irwin R, Madision JM. The Diagnosis and Treatment of Cough. N Engl J Med. 343:1715-1721.
11. Kapoor WN. Syncope. N Engl J Med. 343; 1856-1862.
12. Barbara Turner, Sankey Williams, Darren Taichman, Laurie Kopin, Charles Lowenstein; Dyslipidemia.
Annals of Internal Medicine. 2010 Aug;153(3):ITC2-1.
13. Christine Laine, David R. Goldman, George A. Bray, Jennifer F. Wilson; Obesity. Annals of Internal
Medicine. 2008 Oct;149(7):ITC4-1.
14. Daroff R. Dizziness and Vertigo. Harrisons Principles of Internal Medicine. 17th ed. Ed. Fauci A et al.
New York: McGraw-Hill, 2008.
15. Fields HL and Martin JB. Pain: Pathophysiology and Management. Harrisons Principles of Internal
Medicine. 17th ed. Ed. Fauci A et al. New York: McGraw-Hill, 2008.
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NOTES
Howard S. Smith, MD
Plinio P. Silva, MD, MPH
Intikhab Mohsin, MD
C o n t e n t s
2.2 INTRODUCTION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18
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2.1 Why Pain Management
Though Pain Management is not expressly identified as a content area covered on the
American Board of Internal Medicine (ABIM) certification exam, pain management can be found
throughout many of the medical-content categories, including the 3% of the exam referred to as
Miscellaneous. Beyond exam study, we firmly believe that pain management knowledge is cru-
cially important for healthcare providers in their day-to-day practice.
We have started the chapter with a section on over-the-counter (OTC), Rx, and alternative
pain management. This section will help you understand drug-drug interactions at-a-glance, along
with which OTC analgesics are safe and effective for certain pathologies, diseases, and symptoms.
From there we will dive into an in-depth look at different pain areas.
2.2 INTRODUCTION
The study of pain is defined by the International Association for the Study of Pain (IASP) as
an unpleasant sensory and emotional experience associated with actual or potential tissue
damage, or described in terms of such damage
Acute pain is a physiologic response following a cascade of molecular events initiated by
initial tissue injury
Initially acute pain plays a protective role against further tissue damage, but can progress to
a chronic disease if it persists despite apparent healing
Acute and chronic pain are commonly encountered in clinical practice and present
challenges to the internal medicine physician
The 2011 Institute of Medicine (IOM) report, Relieving Pain in America: A Blueprint for
Transforming Prevention, Care, Education, and Research:
Reported that pain affects as many as 100 million Americans and cost $635 billion
annually
Called for a cultural transformation that would increase awareness and prevention;
improve assessment and management; emphasize patient education; and address
disparities in different patient subgroups
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Further, educate patient regarding the existence of OTC medications that combine
2 or more active ingredients. Patients may not realize that the product contains
1 or more ingredient(s) that they are also taking in the form of another OTC or
prescription medication, which may increase the risk of overdose. To reduce this
problem, you may consider recommending a single-ingredient product whenever
possible
When recommending NSAIDs, inform patients of the following:
Factors associated with the increased risk of NSAID-induced GI complications
are older age, higher daily dosage, history of gastrointestinal or GI ulcer, history
of GI hemorrhage, dyspepsia, NSAID intolerance, use of corticosteroids, use of
anticoagulants, alcohol consumption, and poor general health
Individuals who have severe kidney or liver disease, hypertension, GI ulcers, or who
take certain medications such as anticoagulants should not take OTC NSAIDs in
order to reduce their risk for side effects
Advise patients with peptic ulcer disease to avoid taking NSAIDs for pain relief,
as it increases the risk of gastrointestinal (GI) complications, ranging from mild
dyspepsia to more severe problems such as gastrointestinal hemorrhage
NSAIDs with long-term use may cause renal injury. The use of aspirin, even low-
dose aspirin, can affect renal function in the elderly. Therefore, NSAIDs should
be used with caution in the elderly population because of the higher incidence
of cardiovascular and GI disease, age-related decline in renal function and the
likelihood of polypharmacy
Certain NSAIDs have drug-drug interactions with antihypertensive medications,
which may result in elevated blood pressure and/or decrease in renal function.
Therefore, advise patients with hypertension to avoid NSAIDs. Further, educate
patients with hypertension regarding the use of acetaminophen for pain relief
instead of NSAIDs while using antihypertensive medication. Acetaminophen does
not affect blood pressure or have drug-drug interactions with antihypertensive
medications
Ibuprofen and naproxen sodium may reduce the rate of renal clearance of lithium,
increasing serum levels of lithium and an individuals risk for toxicity
Aspirin may potentiate hypoglycemic effects of sulfonylureas
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Table 2.2: OTC Pain Relievers With Certain Symptoms, Diseases, and Conditions*
Oral anticoagulant therapy Acetaminophen or non-aspirin salicy- Use with caution: Aspirin,
lates (patients taking anticoagulants ibuprofen, naproxen sodium
who also take acetaminophen regu-
larly or at higher doses should be
monitored)
Osteoarthritis or tendonitis Acetaminophen or ibuprofen N/A
Peptic ulcer disease Acetaminophen Use caution with: All NSAIDs
Renal impairment Acetaminophen Use caution with: All NSAIDs
Rheumatoid arthritis Acetaminophen, NSAIDs like N/A
ibuprofen and naproxen sodium
Urticaria, Rash, Pruritic Rash Acetaminophen Salicylates
Adapted from: APhA. Self-Limited Pain Protocol Panel. APhA drug treatment protocols: self-care of self-limited pain. J Am
Pharm Assoc. 1999;39:321-330.
*Labeled dosage should always be recommended unless otherwise suggested by the physi-
cian. Please refer to the Table on Drug-Drug Interactions of Common OTC Analgesics for addi-
tional information.
Imaging
Controversial and generally not helpful for diagnosis
Indicated for patients with red flags on general history or physical exam
Consider in the following settings:
Poor response or worsening in setting of appropriate therapy
New onset of headache in patients at extremes of age
Recent change in quality, severity, or timing of chronic headache
Association with exercise or other factors that would increase intracranial pressure (ICP)
Migraine
High morbidity in general population
Usually unilateral, gradual in onset, throbbing, lasts hours to days, and associated with
significant impairment in function
Aura (often visual), nausea, and vomiting are generally observed
Photophobia and phonophobia are often presentpain is usually relieved by removal of
these stimuli
History alone is often enough to establish diagnosis
Acute Treatment for Migraine
Trigger identification and avoidance
Initial trial with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen at
labeled dosage
If poor response, triptans and ergots are appropriate options
If nausea and/or vomiting is severe enough to limit oral therapy, sumatriptan can be
given subcutaneously or intranasally
Early treatment may lead to improved outcomes
Prophylactic Treatment for Migraine
Appropriate in patients with frequent attacks, long lasting attacks, and attacks associated
with profound functional disability
Improves control of migraine episodes
Lowers incidence of analgesic rebound headache
General classes used for prophylaxis:
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Antihypertensives (-blockers, ACE-I, calcium channel blockers)
Antidepressants (amitriptyline, venlafaxine)
Anticonvulsants (topiramate, valproate, gabapentin)
Avoid opioids for prophylaxis given higher associated risk of chronic daily headache,
dependence, and abuse potential
Patients with frequent episodic TTH are at high risk for developing medication overuse head-
ache (MOH). Limit medication use and consider prophylactic therapy in the setting of frequent
episodes.
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Lamotrigine, topiramate, or gabapentin
If secondary cause ruled out, then a diagnosis of primary thunderclap headache can be con-
sidered.
In addition to standard clinical evaluation, studies used to help elucidate the etiology of TCH
include: Computerized axial tomography (CT) scan, lumbar puncture, magnetic resonance imag-
ing (MRI), magnetic resonance angiography (MRA), and angiography.
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Red flags associated with systemic disease: Older age, cancer history, weight loss, pain >30
days, or poor response to therapy, urinary and stool incontinence
Imaging is usually not helpful early in course of disease, as most episodes are self-resolving
Imaging is indicated if pain persists for >4 to 6 weeks
Plain film of spine should be done first, followed by CT/MRI if necessary
CT/MRI helpful if pain persists for >12 weeks or if there is a high suspicion of systemic
disease (especially cancer and red flags listed)
Treatment of Low Back Pain
Acute Low Back Pain
Usually has an excellent prognosis
Avoid bed rest
Treatment: Reasonable first-line analgesic choice is acetaminophen to help treat
symptoms and improve mobility. NSAIDs may also be considered, but there is
an associated higher incidence of side effects (see OTC, Rx, and Alternative Pain
Management). Muscle relaxants such as cyclobenzaprine can also be considered
If pain persists, initial therapy with opioids can be helpful; however, short course of
scheduled around the clock therapy is preferred over an as-needed (PRN) basis
Subacute and Chronic Low Back Pain
Evaluate for cause based on history and clinical assessment as outlined above
Avoid bed rest
Treatment with NSAIDs or acetaminophen during exacerbation
Limit opioids to short courses of scheduled therapy and to patients with low abuse
potential
Assess and treat exacerbating factors and co-morbid conditions: Psychosocial stress,
depression, and anxiety
A trial of tricyclic antidepressants may be of benefit even for those not suffering with
depression
Exercise has been shown to be beneficial, including yoga, aerobic, and physical therapy
Surgery generally not recommended. Early benefits usually seen but these usually do not
persist
Interventional pain procedures such as nerve blocks, Epidural injections, Sacro-Iliac joint
injections, Facet nerve blocks, Radio frequency ablation etc.
Neck Pain
Spontaneous or traumatic in origin
Usually secondary to cervical spine disease or soft tissue injury:
Myofascial pain
Cervical radiculopathy with or without spinal stenosis
Axial pain or facet joint arthrosis
Red flags: Fever, cancer history (with or without metastasis), and rapid progression of
symptoms
Rule out neurologic compromise by physical examination
Multifaceted therapy is best approach:
Pharmacotherapy: Acetaminophen or NSAIDs are first-line treatments
Muscle relaxant can be added if spasms are evident
Physical therapy with postural correction and rest
Elimination of ongoing stress or inciting factors
Shoulder Pain
Common complaint that affects majority of adults at some point in life
Deep, ache-like pain on lateral arm at point of deltoid insertion (referred pain)
Also associated with difficulty abducting arm above level of the head, pain with pressure on
shoulder area (sleeping on affected side), and reaching towards their back
Important to evaluate for history of trauma, overuse, or other inciting factor
All patients should be evaluated with a plain film radiograph
Traumatic injury should be treated with reduction (closed or open) and immobilization
Specific therapy depends on diagnosis
Non-traumatic injury can be treated with:
Rest and NSAIDs or acetaminophen for symptomatic relief
Gentle exercises followed by strengthening exercises and possible physical therapy
Differential Diagnosis
Fracture
Dislocation
Impingement
Acromioclavicular arthritis
Biceps tendinitis
Rotator cuff tear
Instability
Superior labrum anterior posterior lesion
Frozen shoulder
Cervical disk
Elbow Pain
Presentation depends on etiology and final diagnosis
Most common diagnosis and associated presentations
Epicondylitis (lateral vs. medial)
Local tenderness at muscle origin and/or tendon
Pain worse in morning and associated with tearing sensation
Secondary to avascular necrosis of common extensor or flexor tendon
Treatment: Rest, ice, splinting, NSAIDs or acetaminophen, possible steroid injection,
possible surgery
Ulnar neuropathy
Elbow pain and tenderness that is medially located (where ulnar nerve courses
medial epicondyle)
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Associated with hypoesthesia in the ulnar nerve distribution (medial half of 4th digit
and the 5th digit), can progress to hand weakness secondary to intrinsic muscle
atrophy
Treatment: Rest, therapy, gliding of ulnar nerve
Ulnar nerve subluxation
Snapping sensation with elbow flexion and extension
Paresthesias, especially shock like sensation, in ulnar distribution
Treatment: Rest, flexion avoidance, and possible surgery if it persists
Radial nerve/peripheral interosseous nerve compression
Deep, ache like pain, distributed over extensor surface of forearm
Treatment: Rest, therapy, gliding of radial nerve, and surgical decompression may
be needed
Elbow instability
Traumatic dislocation or repetitive injury (exercise)
Treatment: Physical therapy
Fracture
Associated with acute trauma
Treatment: Reduction and immobilization, possibly open reduction and internal
fixation (ORIF)
Synovitis
Associated with elbow swelling and painful flexion/extension
Treatment: Rest, ice, splinting, NSAID therapy, steroid injection, possible surgery
Arthritis
Trauma or history of inflammation (rheumatoid arthritis)
Treatment: Rest, ice, splinting, acetaminophen as first-line therapy before trying
NSAIDs, possible steroid injection, possible surgery
Olecranon bursitis
Inflammation of olecranon bursa
Localized swelling on posterior forearm at elbow
Treatment: Usually self-limiting so observation is warranted, antibiotics are
indicated if cellulitis is present, incision and drainage (I&D) with bursectomy if no
improvement or systemic symptoms are seen
Hip Pain
Trochanteric Bursitis
Inflammation of trochanteric bursa
Presents as lateral pain that is worsened by pressure/palpation
Treatment: Localized physical therapy, NSAIDs, and rest
Hip Arthritis
Generally presents as pain radiating to groin that is worsened by movement of hip joint
and relieved by rest
Eventually leads to decreased range of motion
Can be caused by many factors, such as osteoarthritis, osteonecrosis, trauma, sepsis, and
rheumatoid arthritis
Treatment
Initially conservative with rest, physical therapy, acetaminophen or NSAIDs, and
physical support (i.e., cane in opposite hand)
obesity is present, weight loss can significantly improve pain
If
Surgical treatment with total joint replacement may eventually become necessary if
pain is refractory to conservative measures
Avascular Necrosis (AVN)
Etiology is generally unknown
Associated with alcohol intake, steroid use, trauma, and sickle cell disease (S or SC)
Presents as groin pain and/or a limp
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Treatment largely surgical, although initially analgesics and physical support can be
helpful
Occult Hip Fracture
Usually secondary to osteoporosis
More common in women
Generally associated with previous trauma (even if minor)
Presents as hip/groin pain and inability to bear weight on affected side
Treatment is surgical stabilization of femoral head and treatment of osteoporosis to
decrease likelihood of future fractures
Malignancy
Presents as constant pain generally not associated with activity
Can be site of primary malignancy or metastasis (especially in patients with a history of
cancer)
Treatment: Surgical intervention may decrease likelihood of future fractures
Infection
Presents with constant joint pain associated with decrease in movement of joint, inability
to bear weight, and generally worse at night
Fevers and history of bacteremia are helpful in making diagnosis
Generally presents with elevated white blood cells (WBC), c-reactive protein (CRP), and
erythrocyte sedimentation rate (ESR)
Aspiration of joint effusion can aid greatly in diagnosis with analysis of aspirate
Treatment: Intravenous (IV) antibiotics and pain relief
Knee Pain
Patellofemoral Syndrome
Presents as anterior knee pain exacerbated by prolonged sitting
Secondary to trauma, overuse or poor alignment of patella and femur resulting in
repetitive irritation
Treatment
Analgesic medication, commonly restricted to acetaminophen or aspirin, for 3 to 4
weeks, followed by a taper
For refractory pain, corticosteroid injections and surgery are options
Pes Anserine Bursitis
Presents as anteromedial knee pain
Usually caused by an abnormal gait or trauma resulting in inflammation of the bursa
between the pes anserine tendons and the medial collateral ligament (MCL)
Treatment
Address abnormal gait, ice, and avoidance of knee flexion. NSAIDs can be used for
pain but low vasculature in this region may prevent adequate therapeutic effect
pain persists for more than 6 to 8 weeks, local anesthetic and methylprednisolone
If
injections are used second as second-line
Prepatellar Bursitis
Presents with anterior knee pain and a swollen knee
Often caused by trauma or after prolonged and frequent pressure, such as kneeling
Can also be caused by urate crystal accumulation
Knee range of motion is generally preserved
Remember: Acute knee pain secondary to trauma can be a result of sports injuries to anterior
cruciate ligament (ACL), posterior cruciate ligament (PCL), medial collateral ligament (MCL), lat-
eral collateral ligament (LCL), or meniscus. In addition, the differential of acute knee pain includes
septic arthritis, gout and pseudogout.
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Nerve compression can occur before or after terminal branch division
Usually presents with medial mid foot pain
Roughly half of patients may have a positive Tinels sign
Compression of the lateral plantar nerve occurs most often (e.g., Joggers foot) and
causes stabbing pain in the medial sole when walking or running
Treatment
Conservative: NSAIDs, icing, massage, shoe modification, steroid injections
Surgical release
Arthritis
Inflammatory or osteoarthritis
Rheumatoid arthritis affecting the foot and ankle can be seen in up to 90% of cases
Gout and pseudogout are commonly seen involving the foot
Treatment may include: NSAIDs or acetaminophen, colchicines, steroid injection
Osteoarthritis of foot and ankle usually involves the first MTP joint and naviculocuneiform,
intercuneiform, and metatarsocuneiform joints
Treatment: NSAIDs or acetaminophen and shoe modification
Plantar Fasciitis
Inflammation of foot plantar fascia
Chronic plantar fasciitis is usually characterized by marked collagen degeneration
Pain and tenderness may be maximal over the origin of the plantar fascia on the medial
calcaneal tuberosity and along the fascia 1 cm to 2 cm distal to the origin
Associated with obesity, foot deformities, and middle age
Typically presents with pain at the distal portion of the heel pad (calcaneus) that is worse
in the morning or when standing after rest
Treatment modalities vary and include: Ice, rest, casting, NSAIDs or acetaminophen,
possible steroid injections, physical therapy, and orthotics
Most cases resolve after a period of conservative treatment
Fibromyalgia Syndrome
Pain-amplification syndrome in highly sensitive patients
Both sensory abnormalities and psychosocial exacerbating factors have been described
2010 PRELIMINARY DIAGNOSTIC CRITERIA (EXCERPT) AS PER AMERICAN COLLEGE OF
RHEUMATOLOGY (ACR) includes a patient satisfies diagnostic criteria for fibromyalgia if
the following 3 conditions are met:
Widespread pain index (WPI) 7 and symptom severity (SS) scale score 5 or WPI 3 -
6 and SS scale score 9
Symptoms have been present at a similar level for at least 3 months
The patient does not have a disorder that would otherwise explain the pain
Treatment
Medication based treatment
Tricyclic antidepressants
Selective serotonin reuptake inhibitors (SSRIs) and serotonin-norepinephrine
reuptake inhibitors (SNRIs)
Lidocaine trigger point injection
Pregabalin and gabapentin
Muscle relaxant
Non medication treatment
Low to moderate intensity exercise has been shown to improve physical symptoms
and functional capacity
Extensive patient education is also beneficial
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2.7 ACUTE AND CHRONIC PAIN IN DIFFERENT PATIENT
POPULATIONS
Women and Pain
Women may have higher prevalence of chronic pain conditions
Evidence suggests that the pathophysiology and experience of chronic pain in women is
different than chronic pain in men. This is thought to be secondary to the expression of
different pain receptors
Spinal cord Mu opioid receptor and Kappa opioid receptor heterodimers may represent
unique targets for providing analgesia in women
Rates of oral and or facial pain, migraines, and fibromyalgia are higher in women when
compared to rates of the same conditions in men
Pathologies specific to women
Menstrual migraines
Chronic pelvic pain
OTC treatments
NSAIDs for short-term treatment reduce swelling and relieve pain
Non-opioid analgesic acetaminophen reduces fever, relieves pain due to headaches,
back pain, sore muscles, and joint pain; can be used in combination with opioid
medications
Pregnancy and OTC analgesics
Acetaminophen: 1st/2nd/3rd trimesters - Category B
Non-salicylate NSAIDs: 1st and/or 2nd trimester - Category B; 3rd trimester Category D
Aspirin should generally be avoided
Analgesics should be used in the smallest, most effective dose for pain relief
Breastfeeding
Acetaminophen and non-salicylate NSAIDs are considered safe
Salicylates can be excreted in human milk
The use of high doses of aspirin can produce effects in the nursing infant
Ethnic Differences
Significant disparities exist on the treatment of pain in minority groups
Minority groups are less likely to be prescribed opioid therapy for severe pain
Minority patients are at risk for under-treatment of acute pain across life span and
treatment settings
They receive less analgesic therapy in general
Pain management and assessment have also been found to be less than adequate for
minority groups in emergency department settings
Different ethnic groups may have slightly different expression of drug metabolizers leading
to higher or lower drug levels than those seen in other patients
This is particularly important with codeine, as a patient who has decreased expression
of the CYP2D6 will not be able to convert codeine (pro-drug) to morphine, its active
metabolite. Therefore these patients will not receive appropriate therapeutic benefit
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Elderly may be more sensitive to higher doses of opioids and exercising caution with slow
increase in doses is safer
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26. Emedicine-Medscape. Iliotibial Band Friction Syndrome Treatment and Management. Available at:
http://emedicine.medscape.com/article/1250716-treatment. Accessed September 17, 2012.
27. Emedicine-Medscape. Prepatellar Bursitis Medication. Available at: http://emedicine.medscape.com/
article/309014-medication. Accessed September 17, 2012.
28. Emedicine-Medscape. Pes Anserine Bursitis Treatment and Management. Available at: http://emedi-
cine.medscape.com/article90412-treatment. Accessed September 17, 2012.
29. Nursing Times. Swift A. Osteoarthritis 2: pain management and treatment strategies. Available
at: http://www.nursingtimes.net/osteoarthritis-2-pain-management-and-treatment-strategies/50.
Accessed September 16, 2012.
30. Merck Manual. Nerve compression Syndromes. Available at: http://www.merckmanuals.com/profes-
sional/print/musculoskeletal_and_connective_tissue. Accessed September 17, 2012.
31. Emedicine-Medscape. Trochanteric Bursitis Medication. Available at: http://emedicine.medscape.
com/article/87788-medication. Accessed September 17, 2012.
32. American Academy of Orthopaedic Surgeries. Ulnar Tunnel Syndrome of the Wrist. Available at:
http://orthoinfo.aaos.org/topic.cfm?topic=a00025. Accessed September 16, 2012.
33. American College of Rheumatology. Carpal Tunnel Syndrome. Available at: http://www.rheumatol-
ogy.org/pracice/clinical/patients/diseases_and-conditions/carpaltunnel. Accessed September 16,
2012.
34. Emedicine-Medscape. Avascular Necrosis Treatment and Management. Available at: http://emedi-
cine.medscape.com/article/333364-treatment. Accessed September 16, 2012.
35. Brandt KD, Mazzuca SA, Buckwalter KA. Acetaminophen, like conventional NSAIDs, may reduce
synovitis in osteoarthritic knees. Rheumatology. 2006;45(11):1389-1394.
36. Emedicine-Medscape. Patellofemoral Syndrome Medication. Available at: http://emedicine.med-
scape.com/article/308471-medication. Accessed September 16, 2012.
37. Feleus A, Bierma-Zeinstra S, Miedema H, Verhaar J, Koes B. Management in non-traumatic arm, neck
and shoulder complaints: differences between diagnostic groups. Eur Spine J. 2008;17(9):1218-1229.
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NOTES
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NOTES
Sarah Boutwell, MD
Dary Costa, MD
Jastin Antisdel, MD
C o n t e n t s
3.2 TINNITUS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
3.6 EPISTAXIS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
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3.1 EVALUATION OF HEARING LOSS
History
Hearing loss is a common complaint, especially in the elderly. Determining the cause of the
hearing loss (conductive, sensorineural or mixed) may direct treatment
The most common causes of conductive hearing loss are:
Otosclerosis (stiffening of the ossicles)
Cerumen impaction
Otitis media
Sensorineural hearing loss is frequently due to presbycusis (high frequency hearing loss of
aging) or noise exposure. An audiogram will determine the severity and type of hearing loss
by measuring the softest sounds (in decibels) the patient can hear. A difference between
the air conduction thresholds and bone conduction thresholds suggests a conductive
hearing loss. Bone and air thresholds are usually the same in patients with sensorineural
hearing loss
3.2 TINNITUS
The sensation of sound in the ears, such as ringing or roaring, without external stimulus. It
may accompany hearing loss
Cause
Often caused by damage to the stereocilia, which can release an electrical signal to the
auditory nerve that is interpreted as sound. Damage can be caused by age related changes
or exposure to loud noise. Objective or pulsatile tinnitus requires imaging studies of the
temporal bone for evaluation
Treatment
Treatment is aimed at reducing annoyance caused by tinnitus and not resolution. It is
often masked by using background noise, such as using a hearing aid to introduce a more
pleasant noise into the ear
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3.3 OTITIS MEDIA AND OTITIS EXTERNA
Otitis Media
An inflammatory process of the middle ear. It can be classified into acute otitis media
(AOM), which can be bacterial or viral and has associated symptoms of fever or pain. Otitis
media with effusion (OME) is middle ear fluid without fever or pain
Etiology
The most common bacterial causes of AOM are Streptococcus pneumonia, Haemophilus
influenza, and Moraxella catarrhalis. OME can develop after an episode of AOM
Presentation
AOM presents with otalgia, fever, and bulging tympanic membrane. OME includes
persistent hearing loss and dull or immobile tympanic membrane
Treatment
AOM is treated with antibiotic therapy, although observation for fever or progressive
symptoms is acceptable for 2 days. Amoxicillin is generally the 1st choice, although
ampicillin, amoxicillin-clavulinic acid, or trimethoprim/sulfizoxazole are also acceptable
treatments. OME is managed by observation or nasal decongestants. If middle ear fluid
persists then tympanostomy tubes can be considered. Untreated AOM will rarely extend
into the surrounding soft tissue or the central nervous system leading to meningitis or
intracranial abscess
Otitis Externa
A painful inflammation of the ear canal with purulent ear drainage. Malignant otitis externa
is a severe form usually in immunocompromised or diabetic patients that leads to invasion
of adjacent bone
Etiology
Pseudomonas aeruginosa is the most common cause of acute otitis externa, although
other aerobic Gram Negative species or funguses are other common causes
Presentation
Acute otitis externa (AOE) presents with fever, irritability, and acute pain, with inflamed
ear canal and tenderness of the ear. AOE is commonly called swimmers ear that is
contracted from swimming in water contaminated with aerobic Gram Negative species
Treatment
The commonly indicated bacteria are sensitive to acidic environments so that topical
agents with low pH, such as in acetic acid solutions, are most effective. Topical antibiotic
solutions may also be used. If the canal is swollen shut, a small foam wick may be placed
in the ear canal to allow penetration of the antibiotic drops
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Treatment
Most cases of acute sinusitis improve without antibiotics. Generally supportive care with
decongestants, nasal glucocorticoids, and nasal lavage is preferred in patients with mild
to moderate symptoms
Adult patients who do not improve after 7 days or who have severe symptoms should be
started on antibiotics. However, patients with mild to moderate symptoms and duration of
symptoms less than 7 days showed that antibiotics had no significant impact on cure rates
Treatment targeted at the most common pathogens includes antibiotics such as
amoxicillin and augmentin
If severe disease develops or intracranial complications occur, patients should undergo
surgical intervention and IV antibiotic administration
Chronic Sinusitis
The presence of sinus inflammation symptoms lasting more than 12 weeks
It is increasingly thought that underlying inflammation is the cause of chronic sinusitis
more than it being primarily an infectious process
The natural history of the illness is constant nasal congestion and sinus pressure with
intermittent flares
Patients should have a CT sinus to evaluate for possible structural obstructions such as
polyps. Patients should also be evaluated by an otolaryngologist to visualize the sinuses
and collect direct cultures
Antibiotic treatment is directed by culture data and is often a prolonged 3 to 4 week
course; however, appropriate treatment with concurrent topical/oral glucocorticoids and
mechanical irrigation is important
Surgery may be indicated if therapy fails
Allergic Rhinitis
After exposure to an allergen, patients develop allergic rhinitis symptoms
Approximately 20% of the North American population is affected and may occur in
seasonal variations
It is more common in people with a family history or individuals with a personal history of
asthma, eczema, or urticaria
Symptoms generally present before 40 years of agesymptoms will gradually decline with
age
Etiology
Allergic rhinitis is a Type I allergic reaction with mast cell degranulation. Degranulation
occurs after the patient is sensitized by as antigen, develops B-cell memory, and then has
a re-exposure. IgA in the mucosal surface is responsible in large part for the rhinorrhea
present in allergic rhinitis. The most typical pollen allergens are caused by trees, grasses,
and weeds that depend of wind for pollinationthese are typical causes of seasonal
allergies. Perennial allergies can be caused by animal dander, cockroaches, mold, dust,
and dust mites
Presentation
Patients present with episodic sneezing, rhinorrhea, nasal passage obstruction, itching of
the eyes, nose, or throat, and tearing of the eyes. On exam the nasal turbinates appear
boggy and pale, conjunctiva is injected, and oropharynx unremarkable. Nasal polyps are
sometimes noted
Pharyngitis
Sore throat caused by either a viral or bacterial infection
One of the most common presenting complaints in primary care
Etiology
Pharyngitis is a typical-presenting symptom for respiratory viruses, and viruses are the
most common cause of pharyngitis
Rhinoviruses account for approximately 20% of all acute pharyngitis cases and 5% of all
cases are caused by coronaviruses
Other frequent viral pathogens include influenza, parainfluenza, and adenovirus
Less common viral causes of pharyngitis include herpes simplex virus 1 and 2,
cytomegalovirus, Epstein-Barr virus, and acute HIV infection
Bacterial pharyngitis accounts for approximately 5 to 15% of all acute pharyngitis cases
Most cases in adults (5 to 15%) is Group A hemolytic Streptococcus pyogenes, which is
associated with rheumatic fever and acute glomerulonephritis
Other causes of strep pharyngitis include Group C and G Streptococcus infections, which
are non-rheumatogenic
Fusobacterium necrophorum is almost as common as strep pharyngitis and can cause
Lemierres disease, an infectious thrombophlebitis of the jugular vein
Depending on a patients exposure, other bacterial agents should be considered such as
Neisseria gonorrhoeae, Corynebacterium diptherieae, Corynebacterium ulcerans, Yersinia
enterocolitica, Treponema pallidum (as seen in secondary syphilis), Mycobacterium
pneumonia, and Corynebacterium pneumoniae
Presentation
In a viral URI setting, acute pharyngitis, as caused by rhinovirus or cornovirus, is usually
mild and associated with other nonspecific URI symptoms such as rhinorrhea, congestion,
and cough
Viral pharyngitis is typically not associated with fever, tender cervical adenopathy, or
pharyngeal exudates
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Viral pharyngitis can be severe when caused by influenza or adenovirus and will be
associated with fever, myalgias, headache, and cough
Adenovirus may additionally be associated with conjunctivitis in about 1/3 to 1/2 of
patients infected
More rare causes of viral pharyngitis can be distinguished by physical exam findings
HSV infection has vesicles and shallow ulcers on the palate, as well as pharyngeal
exudates and inflammation
The small vesicles associated with Coxsackie virus are found on the uvula and soft palate
and form small white ulcers after rupture
Both EBV and CMV can have exudative pharyngitis with fever, fatigue, lymphadenopathy,
and enlarged spleen making it difficult to distinguish clinically from acute Streptococcal
pharyngitis
In the setting of acute HIV infection, patients frequently develop a flu-like prodrome with
myalgias, arthralgias, malaise, fever, pharyngitis, and sometimes a maculopapular rash
The presentation of bacterial Streptococcal pharyngitis is similar in group A, C, and G with
a wide variance in severity of symptoms. Patients present with fever, chills, and abdominal
pain in addition to the pharyngeal pain, but no cough
On exam there is tonsillar enlargement and exudates with tender cervical
lymphadenopathy
The Centor Criteria are typically used to distinguish Streptococcal pharyngitis and
determine antibiotic usage. Some strains of Streptococcal pyogenes can present with
a strawberry tongue and erythematous rash
Other bacterial causes of pharyngitis typically present with exudates but generally
are non-specific in their clinical findings and must be distinguished based on history
alone
Treatment
Determined by the etiology. Viral pharyngitis is managed by supportive care. However,
bacterial often requires antibiotic therapy
Throat swab culture is the gold standard for diagnosis, but rapid-antigen testing can
have up to a 90% specificity, although sensitivity is only 65 to 90% depending on clinical
presentation and collection technique
The Centor Criteria is used to raise the sensitivity of testing and determine
appropriateness of antibiotic usage. A score of less than 2 points directs against antibiotic
usage. A score of 2 to 3 points should receive a throat swab and antibiotic usage dictated
by results of culture data. Scores greater than 3 points should empirically be started on
antibiotic therapy
3.6 EPISTAXIS
Sinusitis
This is a common primary care problem of bleeding from the anterior nasal cavity, usually
unilaterally. Posterior or bilateral bleed is a matter of medical urgency and should be
evaluated immediately
Etiology
Most commonly caused by nasal trauma such as nose picking, foreign bodies, or nose
blowing. Other causes include rhinitis, dry air, nasal septum deviation, atherosclerotic
disease, hypertension, neoplasia, cocaine abuse, alcohol use, and Osler-Webb-Rendu
syndrome (hereditary hemorrhagic telangectasias)
A less common cause is anticoagulation
The most common location is in the anterior septum in a confluence of superficial veins
called Kiesselbach plexus
Presentation
Bleeds are typically unilateral and self limiting. Patients with epistaxis typically have higher
blood pressures, but values usually return to normal once bleed has resolved. Laboratory
values such as platelet counts and INR can be useful in evaluating cause of an acute bleed
Treatment
Direct pressure on the site of the bleed for 15 minutes by compression of nares treats the
majority of cases
Nasal packing or pneumatic sealant can also be used to compress bleeding sites difficult
to compress externally
Sitting upwards reduces venous pressure, and leaning forward decreases the amount of
blood swallowed
A topical decongestant such as phenylephrine acts as a vasoconstrictor. Topical cocaine
can also be used as both vasoconstrictor and anesthetic. Silver nitrate can be applied
topically to cauterize the bleeding site or a patch can be applied. Before
applying silver nitrate, numb the nose bleed with lidocaine
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If bleeding continues or bleed originates in the posterior nasal cavity, an otolaryngology
consult for packing and hospitalization for monitoring and pain control is advised
In the case of life-threatening hemorrhage, surgical intervention such as ligation of the
nasal arterial supply or endovascular embolization may be necessary
Antibiotic coverage for staphylococcal infection is necessary while packing is in place
Oral Tumors
Include tumors from the lips, the hard and soft palate, the tongue, salivary glands, or even
the gingiva and oral cavity floor. The anterior 2/3s of the tongue and the floor of the mouth
are the most common location for oral tumors
Dental Infection
Dental caries and periodontitis are the sequelae of plaque build-up and bacterial infection
leading to the destruction of involved tissues. Plaque is a biofilm composed largely of
normal oral bacteria
Dental Caries
Defined as inclusions in the mineralized tooth from acidic byproducts of bacterial
metabolism
Caries progress inward from the surface, eventually involving the dentin and then the
pulp from which infection can extend into the periodontal tissues at the root
Streptococcus mutans is the dominant organism causing caries, but other organisms such
as Lactobacillus acidophilus, Streptococcus salivarius, and actinomyces also contribute to
the formation of dental carries
Monosaccharides and disaccharides such as glucose, sucrose, lactose, and maltose
provide appropriate substrate for glycolysis and contribute to bacterial acid production
Bacteria can utilize sucrose to synthesize polyglycans that increase adherence and
aggregation of bacteria on the tooth surface. This polyglycan can also be utilized
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by bacteria as a food source when dietary food sources are absent. This prolongs
acid production beyond the period of substrate clearance, increasing the chance of
developing dental caries
Complications of dental caries include extension of infection into the pulp, necrosis of the
pulp, extension into the root canal and the periapical area of the periodontal ligament
Further involvement can result in periapical abscess, nonsuppurating inflammation, or
cyst. If the infection goes unchecked, it may result in cellulitis, osteomyelitis, or septic
thrombophlebitis (Lemierres disease)
Incidence of new dental caries can be affected by 50 to 60% via systemic ingestion of
fluoride or 35 to 40% by topical application
Periodontal disease includes gingivitis and chronic periodontitis, both of which are caused
by plaque build-up
Gingivitis represents an earlier, reversible form of chronic periodontitis. Gingivitis refers to
inflammation of the gingiva, or gum line. Onset of disease occurs within 2 weeks of poor
tooth hygiene
Chronic periodontitis, the progression of gingivitis, with permanent bone resorption leads
to the loss of tooth support
Abscess can also develop when chronic inflammations flare at the neck of the tooth in a
single location. It accounts for the majority of tooth loss in people older than 35 years
With periodontal disease there is evidence of inflammatory infiltrate into the gingival, but
no direct invasion by bacteria in the early stages
Gram Negative anaerobic bacteria are predominantly responsible for periodontal disease.
Infections are typically microbial and include Porphyromonas gingivalis and Treponema
denticola, which grow synergistically
Halitosis
A foul odor that comes from the nasal passages or oral cavity
The bacterial decay of food and cellular debris produces sulfur compounds creating the
odor
Halitosis is associated with periodontal disease, dental caries, acute gingivitis, oral
abscess, and tongue coating
Less common causes include esophageal diverticulum with retained food particles,
esophageal stasis, sinusitis, lung abscess, and pockets of decay in tonsillar crypts
There are also systemic diseases that can cause halitosis such as renal failure (ammoniac),
hepatic failure (fishy), and ketoacidosis (fruity)
Helicobacter pylori infection can also create ammoniac breath
Treatment is aimed at correcting poor hygiene including tongue brushing and treating
infection
Tongue Syndromes
Glossitis
A red, smooth surfaced tongue caused by inflammation and subsequent loss of filiform
papillae. Typically non-painful, it is often associated with nutritional deficiencies such as
niacin, riboflavin, iron, or vitamin E. It may also be caused by drug reactions, dehydration,
foods, and even autoimmune reactions. If no cause is identified, empiric nutritional
replacement therapy is often the best first-line therapy
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Presentation
Patients typically present with jaw, face, and head pain. They may have difficulty opening
their jaw or experience catching or sticking with accompanying joint popping and clicking
Global headache, shoulder, and neck pain are also common as well as tooth sensitivity,
malocclusion, and abnormal tooth wear
Occasionally tinnitus, dizziness, and hearing loss are noted
CT can show bony tissue degenerative changes while MRI can evaluate for soft and hard
tissue abnormalities
Treatment
Conservative management is the most common method of treatment; less than 5%
of cases require treatment. A combination of self-care, physical therapy, splinting, and
medication with muscle relaxants and NSAIDs can provide significant relief of pain. Short
courses of oral steroids and opiates can be used in patients with moderate to severe pain
who are not responding to initial therapy; joint injections with steroid can also be useful.
Alternative therapy such as massage has also shown beneficial effects
Self-care Recommendations
The rest of the muscles and joints allow healing
Soft food enables muscles and joints to heal
Not chewing gum lessens muscle fatigue and joint pain
Relax your facial muscles: "Lips relaxed teeth apart"
No clenching; it irritates joints and muscles
Yawning against pressure prevents locking open and jaw pain
Moist heat for 20 minutes promotes healing and relaxation
Ice is for severe pain and new injuries (less than 72 hours)
Heat and ice5 seconds of heat, 5 seconds of icefor pain relief
Good posture; avoid head-forward position
Sleeping position: Side lying, with good pillow support
Jaw exercise: Open and close against finger pressure
Exercise: 20 to 30 minutes at least 3 times a week
Acupressure massage between thumb and forefinger
Over-the-counter analgesics
Yoga and meditation for stress reduction
Massage promotes healing and relaxation
An athletic mouth guard can give temporary relief
Avoid long dental appointments
Do not cradle the telephoneit aggravates the neck and jaw
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NOTES
C o n t e n t s
4.5 GLAUCOMA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64
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4.1 BASIC ANATOMY AND FUNCTION
Eyelids: Provide protection to the eye. They evenly spread the tear film on the ocular
surface. Eyelid margins carry meibomian glands, which provide an important component of
the tear film. Dysfunction of these glands can cause a form of blepharitis and dry eye
Conjunctiva: Lines the inner aspect of the eyelids and covers the sclera. The conjunctiva
secretes an important component of the tear film and provides the immune surveillance of
the ocular surface
Cornea: It is the transparent avascular anterior surface of the eye. It transmits and focuses
light on the photoreceptors of the retina. Minor irregularities or opacities in the cornea can
significantly affect visual acuity. The cornea is very densely innervated, and thus corneal
diseases such as an abrasion can be associated with severe pain
Anterior chamber angle: It is the angle between the cornea anteriorly and the iris
posteriorly and is responsible for draining out the aqueous humor and thus maintaining a
normal intraocular pressure. Dysfunction in this area can cause a form of glaucoma
Crystalline lens: Together with the cornea, it focuses incoming light on the photoreceptors
of the retina. Contraction of the ciliary muscle surrounding the lens allows accommodation
of the lens, the ability to focus on objects at different distances from the eye
Uvea: Composed of the iris, ciliary body and choroid. The iris forms the pupil, which
continuously controls the amount of light entering the eye similar to the shutter of a
camera. The ciliary body secretes the aqueous humor, which provides nutrition to the
avascular cornea and lens. The choroid provides nutrition to the outer layers of the retina
Retina: Has the photoreceptors, which convert light to electrical impulses. The peripheral
part of the retina provides night vision and visual field. The central part of the retina, the
macula, is the most sensitive part, providing fine central vision
Optic nerve: Carries the electrical impulses from the retina to the brain. The optic nerve
head can be visualized during direct ophthalmoscopy and is seen nasal to the macula. The
optic nerve is surrounded by the 3 meningeal sheaths of the brain and its subarachnoid
space is connected to that of the brain
Orbit: The skull socket, which contains and protects the eye globe, part of the optic nerve,
the extraocular muscles, nerves, and blood vessels. The orbital apertures are continuous
with the intracranial cavity
Vitreous humor
Choroid
Retinal
pigment
epithelium
Macula
Retina
Optic nerve
Fovea
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Astigmatism: Astigmatism occurs when the curvatures of the cornea (or the lens) at
different axes are not equal (the shape of an American football instead of a basketball),
resulting in failure to focus the image on the macula
Presbyopia: Progressive age related diminution of near vision secondary to a decrease in
the amplitude of accommodation. Usually starts in the 4th decade. Vision is fully corrected
by appropriate reading glasses
II- Cataract
Opacification of the crystalline lens.
Types
Senile (age-related) cataract: The most common type
Secondary: May be seen with trauma, corticosteroids, uveitis, radiation, diabetes mellitus, or
systemic diseases such as Wilsons disease or neurofibromatosis type 2
Congenital or developmental: Usually leads to amblyopia and nystagmus
Symptoms
Painless, slowly progressive blurring of vision that is usually bilateral yet often
asymmetrical
Glare (often with bright sun light or automobile headlights at night)
Decreased color vision
Some patients initially notice frequent changes in their eyeglass prescriptions and
improvement of near vision in a phenomenon known as second sight
Signs
Decreased visual acuity
Direct ophthalmoscopy: Clouding of the lens, dim or absent red reflex, difficulty
visualizing the retina
Treatment
Surgical removal of the lens followed by placement of an intraocular lens
Surgery is indicated if the decrease in vision affects a patients ability to perform his/her
activities of daily living. The ophthalmologist may sometimes advise earlier or delayed
intervention depending on other ocular comorbidities
Cataract surgery is usually an outpatient surgery
Types
1- Dry type (atrophic, non-exudative): 90% of cases
Symptoms
Gradual loss of central vision (usually over months or years)
Distortion of vision
An Amsler grid can be used to assess the progression of the disease. It is a grid of
horizontal and vertical lines. A change or distortion in the lines may indicate progression
of the disease, which may require treatment
Signs
Drusen are small yellow or white spots in the macula. They are usually bilateral but may
be asymmetric
In advanced cases, sharply demarcated areas of retinal atrophy (geographic atrophy) are
seen
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2- Neovascular or exudative (wet): 10% of cases
Symptoms
Central visual loss, which may be rapidly progressive
Signs
Macular drusen, retinal pigment epithelial detachment
Choroidal neovascularization with retinal hemorrhages
Subretinal fibrosis and scar
Screening
A comprehensive eye exam by an ophthalmologist is recommended every 2 years for
people under age 65 and annually for those over age 65
Prevention
A diet rich in leafy green vegetables may lower the risk of AMD
Cessation of smoking
Protective measures against exposure to excessive sunlight
Control of hypertension
Treatment
Dry AMD:
High dose antioxidant vitamins and minerals (vitamin C and E, beta-carotene, zinc, and
copper AREDS formula): Can decrease the risk of AMD progression in moderate and
advanced AMD. Note that there is an increased risk of lung cancer with beta-carotene in
smokers and ex-smokers
Amsler grid: Should be provided for self-test on a regular basis
Low vision aids
Surgery: Miniature intraocular telescope implantation or macular translocation surgery
may be of benefit
Wet AMD:
Intravitreal injections of Anti-VEGF (vascular endothelial growth factor): The current
standard of care
Intravitreal injections of steroids
Thermal laser photocoagulation or photodynamic therapy (PDT) may sometimes be
used
4.5 GLAUCOMA
Glaucoma is a progressive optic neuropathy characterized by visual field loss and optic
disc cupping. It is usually associated with elevated intraocular pressure, which is the key
modifiable factor
Glaucoma is the 2nd leading cause of legal blindness in the US after AMD and the leading
cause among African Americans
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Symptoms
Haloes around lights, eye pain, and headache
Precipitating factors include being in a dark room, reading, emotional stress, and
medications causing mydriasis such as topical mydriatics, systemic parasympathetic
antagonists, or sympathetic agonists (e.g., cold remedies)
Signs
Reduced vision (often less than 20/200)
Ciliary hyperemia (violaceous circumcorneal injection) and corneal edema
Anterior chamber is shallow
Nonreactive mid-dilated pupil
Intraocular pressure is usually very high (50 to 100mmHg)
Treatment
Immediate referral to an ophthalmologist
Supine position to encourage the lens to shift posteriorly under the influence of gravity to
break the attack
Systemic: Intravenous acetazolamide, mannitol, or glycerol. Analgesia and an antiemetic
may be required
Topical: a-agonists, b-blockers, steroids, pilocarpine
Central corneal indentation with an indentation lens can force aqueous into the angle and
may break an attack
In resistant cases: Emergency laser or surgical iridotomy, lens extraction, or
trabeculectomy
Non-proliferative
Microaneurysms: Tiny red dots in the retina (the earliest sign)
Retinal hemorrhages: Flame-shaped and dot/blot hemorrhages
Exudates: Waxy yellow lesions with relatively distinct margins
Cotton wool spots: Small, whitish, fluffy superficial lesions
Diabetic Maculopathy
Macular edema, exudates or ischemia: The most common cause of visual impairment
Proliferative Retinopathy
Retinal neovascularization: New blood vessels form in response to retinal ischemia. New
blood vessels may bleed spontaneously resulting in retinal and vitreous hemorrhages and
subsequent retinal fibrosis and detachment
Iris neovascularization: May cause occlusion of the trabecular meshwork and neovascular
glaucoma
Screening
A dilated comprehensive eye examination by an ophthalmologist is indicated immediately
after the diagnosis of diabetes type 2 is made. An examination is indicated within 35 years
of the diagnosis of type 1 diabetes
Subsequent examinations for both type 1 and type 2 diabetic patients should be repeated
annually. Examinations will be required more frequently if retinopathy is progressing
Treatment
Improved glycemic and blood pressure control can prevent and delay the progression of
diabetic retinopathy
Laser photocoagulation therapy prevents loss of vision in patients with severe non-
proliferative, proliferative diabetic retinopathy and/or macular edema
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4.7 ACUTE LOSS OF VISION
Transient Loss of Vision (Amaurosis Fugax)
Embolic: Occlusion of blood supply to the retina or the visual pathway (atrial fibrillation,
carotid thrombus)
Vasospasm: Migraine or hypertensive emergency
Hypoperfusion: Low blood pressure, shock, or orthostatic hypotension
Functional disorder: Hysterical and malingering
Treatment
Antiviral eye drops (e.g., topical ganciclovir)
Most uncomplicated cases resolve by 2 weeks
Chalazion
Chronic, sterile, granulomatous inflammation of the meibomian glands of the eyelid. A
secondarily infected chalazion is referred to as an internal hordeolum
Symptoms
Gradually enlarging painless nodule. Painful if infected
Signs
A nodule within the eyelid that may be tender if inflamed
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Treatment
Warm compresses: At least a 3rd of chalazia resolve spontaneously
Persistent lesions may be treated with surgery (incision and curettage) or intralesional
steroid injection
Recurrent chalazia should be biopsied to rule out masqueraders such as sebaceous cell
carcinoma of the eyelid
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4.11 EYE IN RHEUMATOLOGICAL DISEASES
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NOTES
Anne Davis, MD
C o n t e n t s
5.1 SEXUALITY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76
5.2 CONTRACEPTION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
5.3 VAGINITIS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
5.7 DYSMENORRHEA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
5.8 MENOPAUSE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
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5.1 SEXUALITY
Sexual Functioning
Most women describe themselves as heterosexual, about 4% as bisexual, and about 2% as
lesbian; however, about 12% of women report sexual experience with another woman
Most heterosexual women in the US begin having vaginal intercourse around age 17; 98% of
women will have intercourse at least once
Most couples are satisfied with their sexual relationships and report sex from a few times a
week to a few times a month. Even those having sex much less frequently can report high
sexual satisfaction
Important components of the sexual response (desire, arousal, and orgasm) are similar
in women and men, but may not occur in a linear manner. For example, many women
experience arousal before desire
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Table 5.1: Drug Interactions
Medicines that can decrease the efficacy of hormonal
contraception
Griseofulvin
Rifampin
Carbamazepine
Phenobarbital
Dilantin
Oxcarbazepine
Bosentan
Safety
Combined hormonal contraceptives are Safety of contraception for women with medical
much safer than pregnancy. Serious conditions: Where to go to know
complications of use are largely related to
U.S. Medical Eligibility Criteria for Contraceptive Use,
thrombotic effects of estrogens
2010
Users experience a very small absolute Adapted from the World Health Organization Medical
risk of venous thrombosis, embolism, Eligibility Criteria for Contraceptive Use, 4th edition
stroke or myocardial infarction. These risks www.cdc.gov
Barrier Methods
Male latex condoms are widely used for contraception and are easy obtained over the
counter. Polyurethane condoms are as effective as latex versions. Female condoms are also
available
Condom use reduces, but does not eliminate, the risk of STI acquisition
Typically, at least 15% of couples that use condoms as their only method will experience a
pregnancy during a year
Other barrier methods include the female diaphragm and cervical cap. These methods must
be used with a spermicide. Failure rates are high, about 20% per year
Emergency Contraception
Emergency, or post-coital, contraception reduces the risk of pregnancy after intercourse
primarily by inhibiting ovulation. Women over 17 may obtain EC without a prescription
EC contains progestin in a dose higher than OCs, without estrogen, and will reduce the risk
of pregnancy related to unprotected intercourse in the previous 72 hours
A newer form of EC, ulipristal acetate, is effective for 5 days after unprotected sex
A copper IUD is effective for EC up to 7 day after unprotected sex
5.3 VAGINITIS
Women with vaginitis describe symptoms with some combination of abnormal discharge,
irritation or itching, odor and occasionally pain. Some forms of vaginitis are due to sexually
transmitted infections. A directed physical exam and wet preparation should guide diagnosis and
treatment. Asymptomatic yeast or BV detected by PAP testing or exam does not require treatment.
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Bacterial Vaginosis
Symptoms: Discharge, fishy odor especially after intercourse
Pre-disposing factors: Previous BV, tends to recur
Exam: Thin, grey vaginal discharge
Wet mount: + odor with KOH added, Clue cells on saline prep, pH high
Not sexually transmitted
Oral or topical metronidazole, avoid alcohol with oral form
Trichomoniasis
Symptoms: Itching, can be intense
Predisposing factors: New partner
Exam: Greenish, frothy vaginal discharge, erythematous vagina and cervix (strawberry
cervix)
Wet mount: WBCs, motile flagellated trichomonads, pH is high
Treatment: Oral metronidazole 1 dose, also for partner
Sexually transmitted
Herpes
Symptoms: Focal edema, pain and vesicles that ulcerate, primary infection may occur with
fever and adenopathy
Predisposing factors: New partner
Exam: Swollen, tender area on perineum, cervical, labial or perineal blisters or ulcers, vaginal
discharge
Wet mount: WBCs, pH is low
Treatment: Oral acyclovir or valacyclovir
Sexually transmitted, symptoms may be due to primary or recurrent HSV
Atrophic Vaginitis
Symptoms: Painful intercourse, irritation with urination, discharge, itching
Predisposing factors: Menopause
Exam: Thinned vulvar tissues, narrowed introitus, erythema
Wet mount: WBCs
Treatment: Lubricants, systemic or topical estrogen replacement
Intermenstrual Bleeding
Occurs between regular menstrual cycles. Rule out pregnancy. Other causes include
cervical or endometrial polyps, myomas, cervicitis (chlamydial is common), endometrial
hyperplasia or cancer, or cervical dysplasia/cancer
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Heavy Menstrual Bleeding (HMB)
May lead to severe anemia. A complete blood count should be included. Start iron
empirically
Can be due to endocrinopathies, hemoglobinopathies, or anatomic causes
Endocrinopathies include hypothyroidism and PCOS. Von Willebrand disease is the most
common inherited coagulopathy
Anatomic causes include uterine polyps and myomas (especially when impinging on the
uterine cavity-submucous)
Treatment
Amenorrhea and oligomenorrhea treatment Polycystic ovarian syndrome (PCOS)
will depend on the etiology such as correction Associated with infrequent, irregular and sometimes
heavy menstrual flow due to anovulation. Diagnosis:
of thyroid disease and hyperprolactinemia.
Typical ovarian appearance on sonography, clinical evi-
Primary ovarian failure requires hormone dence of hyperandrogenism such as hirsutism and acne,
replacement and careful discussion of fertility with menstrual irregularity. Many patients are also obese
implications. Menstrual irregularities due to and abnormalities of lipids and insulin resistance may be
present. Infertility may occur.
PCOS can be managed with weight loss and
OCs in women not seeking pregnancy
HMB should be treated initially with iron replacement. When not associated with a hormonal
etiology or cancer, HMB can be treated with non-hormonal treatments such tranexamic
acid, or NSAIDs or hormonal treatments such as OCs, DMPA or the hormonal IUD. Surgical
treatments include endometrial ablation, myomectomy or hysterectomy
5.7 DYSMENORRHEA
Dysmenorrhea refers to pain with menstrual bleeding. Primary dysmenorrhea is pain in the
absence of an anatomic cause. Primary dysmenorrhea is very common in young women; 85% of
adolescents experience menstrual pain and 15% describe it as severe.
Primary dysmenorrhea: May be associated with nausea, vomiting, and inability to participate
in normal activities. Related to over-production of uterine prostaglandins. Effective
treatments include NSAIDs, acetaminophen, and hormonal therapy with OCs, DMPA
Secondary dysmenorrhea may also respond to NSAIDs, acetaminophen, and hormonal
treatments; however, when due to another etiology that must be addressed: Myomas,
endometriosis
Ibuprofen
Naproxen sodium
Acetaminophen
Recommend labeled dosing of OTC analgesics
Indications
Symptomatic hot flushes or vaginal dryness, not prevention of heart disease or osteoporosis
Contraindications
Elevated risk of CVA, MI, DVT, current breast cancer any age, active liver disease (local
therapy in vagina likely acceptable risk in any of these conditions)
Risks
Different for women in their 50s and women 60 and older
Younger women estrogen only (no uterus): No increased risk of breast cancer, very
small increase VTE, increased risk of heart disease and stroke very small, if any
Younger women estrogen and progestin (with uterus): Estrogen and progestin very
small increased risk of breast cancer after 4 to 5 years of use. Small increase risk VTE,
risk of heart disease and stroke very small if any
Older women: Increase risk VTE, MI, CVA as well as breast CA. Absolute risks for all
outcomes low but serious outcomes. Higher threshold to start replacement
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NOTES
C o n t e n t s
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6.15 AUTOIMMUNE BLISTERING DISEASES . . . . . . . . . 134
Major features
Pruritus
Facial and extensor involvement in infants and children
Flexural lichenification (increased skin markings) in adults
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Chronic or relapsing dermatitis
Personal/family history of atopy
Eye findings: Keratoconus may be associated, anterior and posterior cataracts (usually
secondary to long term steroid use)
Infections
Eczema herpeticum
Molluscum contagiosum
HPV
T. rubrum and P. ovale
S. aureus in 90%
Impaired innate antimicrobial peptides
Human B-defensin and cathelicidins Decreased in AD patients
May explain increased colonization
Elevated IgE
Most AD patients DO NOT have food allergy
Contact Dermatitis
Florist dermatitis - Compositae Sesquiterpene lactone
Causes: Ragweed, chrysanthemum, feverfew, liverwort, lettuce, sage, artichoke,
marigold, sunflower, philodendron, Peruvian lily, and others
Patch test with Sesquiterpene lactone mix
Chromium
Cement and tanned leather
Blackjack felt dermatitis
Cross-reacts with nickel
Cobalt
Vitamin B12 injections
Cross-reacts with nickel
Colophony Resin from tree sap used for adhesion
Bandages
Turpentine lacquer and varnish
Mascara, chewing gum, and newspapers
Preservatives
Quaternium-15 #1 cause of preservative dermatitis
Imidazolidinyl urea #2 culprit
Pigmented Purpuras
Chronic capillaritis; superficial vessels dilated with endothelial cell hypertrophy and
surrounding extravasated red blood cells
Variants include:
Progressive pigmentary dermatosis of Schamberg
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Lichen aureus
Eczematoid purpura of Doucas and Kapetanakis
Lichenoid dermatitis of Gougerot and Blum
Purpura annularis telangiectodes of Majocchi
Inflammatory disorder that affects the skin, mucous membranes, nails, and hair
The Ps: Purple, polygonal, pruritic, papule, planar
Wickhams striae fine, whitish reticulated networks on surface of well-developed plaques
Prevalence: <1%, no racial preference
Many clinical subtypes:
Annular (more common in darker skin), atrophic, linear, erosive, hypertrophic (shins)
Erosive mucous membrane disease more common in patients with hepatitis C
infection
Graham-Little syndrome
Follicular lichen planus of skin and/or scalp
Multifocal cicatricial alopecia of scalp
Nonscarring alopecia of axillary and pubic areas
LP of the nails
10 to 15% of cases
Usually in combination with other LP lesions on skin
20-nail dystrophy (trachyonychia)
Thinning, longitudinal ridging, and distal splitting of nail plate (onychoschizia)
Also, onycholysis, longitudinal striation (onychorrhexis), subungual hyperkeratosis, or
anonychia
Drug-related
b-blockers
Antimalarials
Captopril
Gold
Penicillamine
HCTZ
NSAIDs
Small (0.5 to 1.5 cm) lesions over upper trunk and proximal extremities
Early age of onset/young adults
Streptococcal throat infection frequently precedes eruption
Generalized pustular
Fever, lasting several days, with eruption of sterile pustules 2 to 3 mm diameter
paralleling the fever
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Fingertips may become anonychia and atrophic
Hypocalcemia, albuminemia, and leukocytosis
Can be induced following cessation of systemic steroids
Psoriatic nail disease
Seborrheic Dermatitis
Related to Malassezia furfur and sebum production
Seen in infants and post-pubertal
Face/scalp/chest/back/intertriginous areas
Salmon colored, waxy scaling patches and plaques
Severe cases in: HIV, Parkinsons
Seborrheic dermatitis like: Langerhans cell histiocytosis
Treatment: Topical steroids, ketoconazole, topical tacrolimus/pimecrolimus
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Pityriasis Rosea
Erythema Multiforme
Target lesion = dusky center, white ring, with surrounding erythema; tend to occur on face,
mucosal, acral sites, genitalia
Note: Steven Johnson syndrome (SJS) and toxic epidermolytic necrolysis (TEN) are
now thought to be variants within a spectrum; erythema multiforme is considered
distinct, though with clinical overlap
Erythema multiforme minor
#1 cause = HSV; outbreak precedes EM by 3 to 14 days
No systemic or mucosal involvement
Erythema multiforme major
Caused by infection in 90% (HSV, Mycoplasma), rarely drugs
Has systemic or mucosal involvement
Full development of all lesions in 24 to 72 hours, last at least a week
Does not progress to TEN
Rowell syndrome
Patients with SLE develop lesions of erythema multiforme
Drug Reactions
Drug eruptions (adults > children)
Drug exanthem
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Hypersensitivity syndromes
Seen most often with anticonvulsants and sulfonamides, and less commonly with
allopurinol, dapsone, and gold
Reactions present with fever, rash with facial edema, eosinophilia,
lymphadenopathy, hepatitis, and nephritis
Pathogenesis of anticonvulsant hypersensitivity is related to the individuals inability
to detoxify arene oxide metabolites of these medications, due to lack of epoxide
hydrolase
Diphenylhydantoin, phenobarbital, and carbamazepine are known to cross-
react, whereas valproic acid generally does not cross react
DRESS (Drug reaction with eosinophilia and systemic symptoms)
Also known as drug/dilantin hypersensitivity syndrome
Most common from aromatic antiepileptic agents
Phenytoin, carbamazepine,and phenobarbital (these 3 cross-react) and the
sulfonamides, allopurinol
Predisposed to by epoxide hydrolase deficiency (anti-epileptics), slow acetylator
(sulfas)
Can be difficult to distinguish from serum sickness
15 to 40 days after exposure
Clinically, facial edema is hallmark
Usually see elevation in LFTs (liver most commonly involved)
Possible role of HHV-6 and 7 has been proposed
Monitor TFTs for 12 weeks; hypothyroidism possible
10% mortality
Acute generalized exanthematous pustulosis (AGEP)
Non-follicular, sterile pustules on erythematous background; ddx pustular psoriasis,
candidiasis
Caused by beta-lactams, macrolides, terbinafine
<4 days after exposure
Can be associated with elevated WBC (18 to 25)
Patch testing positive in 80%
Can see upregulated IL-8 (neutrophil chemoattractant)
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Acne fulminans
Explosive: Trunk > face
Leukocytosis, fever, arthralgias
Lytic bone changes
Sternoclavicular joints and chest wall inflammation
Tx with prednisone and isotretinoin
Chloracne
Malar, postauricular, scrotum
Cutting oils and dioxin
Acne-like induced eruptions
Halogens, bromide, and iodide
Androgenetic hormones such as testosterone, ACTH, corticosteroids
Isoniazid (INH)
Lithium
Erbitux
Phenytoin
Cyclosporine
Vitamins B2, B6, and B12
Treatment
Erythromycin and clindamycin less effective against P. acnes
Tetracyclines below MIC can inhibit PMNs, cytokines and P. acnes production of lipase
Risk of photosensitivity demecycline > doxycycline > tetracycline > minocycline
Unlike other tetracyclines, doxycycline is excreted via the GI tract rather than the
kidneys, and thus is the tetracycline of choice in renal-compromised patients
Minocycline can cause hyperpigmentation of the skin. Three types are generally
described:
Blue-black discoloration: Appearing in areas of prior skin injury, such as acne scars
Blue-gray discoloration: Often at the lower anterior legs and forearms
Muddy brown discoloration: Found on sun-exposed areas. The least common type
of hyperpigmentation
Only rifampin reduces OCP efficacy in studies
Isotretinoin
120-150 mg/kg total dose
SE: Pseudotumor with TCN
Large study failed to show increased risk of suicide
Large study failed to show association with IBD
Hormonal
Estrogen
Ethinyl estradiol most common
Desogestrel, norgestimate and gestodene lowest andro properties
Combo works best
Anti-androgen
Spironolactone
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Otophyma: Earlobes
Blepharophyma: Eyelids
Treatment
Topical
Antibiotics often effective
Topical metronidazole active against papules and pustules, but not telangiectasia
and flushing
Topical sulfur-based preparations
Azelaic acid
Sunscreen
Systemic
Antibiotics generally responds well Tetracyclines
Isotretinoin indicated in phymas; but rosacea often rapidly recurs after
discontinuation of isotretinoin
Hidradenitis Suppurativa
Component of follicular occlusion triad
Acne conglobata, hidradenitis suppurativa, and dissecting cellulitis of the scalp
Associated with smoking and being overweight
Hidradenitis suppurativa severity predictors
Atypical locations more common in men than in women
Men have more severe disease
Increased body mass index
Atypical locations
A personal history of severe acne
Absence of a family history of HS were associated with more severe disease
Clinical: Recurrent, inflamed cystic nodules in intertriginous areas. Chronic inflammation can
lead to the formation of sinus tracts
Treatment: Topical antibiotics (i.e., clindamycin), antibacterial washes, oral antibiotics
(rifampin + clindamycin > tetracycline class), oral retinoids
Figure 6.13: Tinea corporis
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Majocchis granuloma
Indurated, pink plaque with prominent hair follicles caused by T. rubrum
Needs systemic antifungals
T. barbae
In men, the bearded area of the face and neck, generally inflammatory
Associated with exposure to animals
T. cruris
Mainly seen in males, involves the groin, perineal and perianal skin
Direct or indirect contact
T. pedis - 3 clinical subtypes:
Moccasin
Interdigital
Bullous
Interweb infections often involve fungi, yeast, gram negative and positive bacteria
Onychomycosis
Fungal infection of the nails due to dermatophyte, yeast, or nondermatophyte
Clinical subtypes
Distal lateral subungual onychomycosis
Infection begins distally and involves the nail bed, nail plate and lateral nail
fold; thick nail with debris, loose or cracked nail plate
Proximal white subungual onychomycosis
Rarest form of onychomycosis
AIDS marker
Organisms enter the cuticle and infect the proximal part of the nail bed
causing white islands that slowly invade the nail plate
White Superficial Onychomycosis
Organism invades the surface of the nail plate of toenails only
Irregular white chalky opaque patches on the nail
Candidiasis
Candida albicans, C. glabrata (fluconazole resistant), C. parapsilosis, C. tropicalis, C. krusei,
C. dubliniensis (thrush in HIV)
Most common fungal opportunistic infection, may be difficult to evaluate; yeast are
ubiquitous and part of endogenous flora
Prototypically bright red, moist patches with satellite pustules
Intertriginous involvement or thrush
Factors contributing to candida infection:
Impaired epithelial cell barrier, systemic illness, neutrophil and macrophage disorders,
immune disorders, therapeutic agents, congenital or acquired endocrine disorders,
malignancies, indwelling catheters, hyperalimentation, heat, humidity, and friction
Clinical variants
Angular cheilitis: A classic complication of childhood diabetes, presents as a white
curd-like material adherent to red, fissured oral commissures
Median rhomboid glossitis
Chronic paronychia: Involves the proximal nailfold; erythema, swelling, separation
from the nail margin with nail dystrophy
C. parapsilosis
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Bacillus anthracis Spore-forming, gram+ rod
Animal exposure
Exotoxins
Edema toxin = Edema factor (EF) + protective antigen (PA)
EF causes gelatinous edema by increasing camp
Lethal toxin = Lethal factor (LF) + PA
LF can cause shock and death in disseminated disease by release of TNF and IL-1
PA allows entry of exotoxins into the cells
Antibodies to PA will prevent LF and EF action
3 clinical forms:
Inhalational, GI, and cutaneous
Cutaneous
Malignant pustule followed by bullae and eschar
Treatment
ConventionalPCN
Bioterrorismciprofloxacin or doxycycline
Borrelia
Lyme
Tick-borne
Organism: B. burgdorferi
Vector: Ixodes dammini, pacificus, ricinus
Clinical: Erythema migrans, chronica atrophicans (rare, chronic disease resulting in acral
sclerodermoid changes
Treatment: Doxycycline Amoxicillin for kids and pregnant women
Relapsing fever
Louse-borne
Organism: B. recurrentis
Vector: Pediculus humulus
Clinical fever, HA, systemic disease
Treatment: Doxycycline
Relapsing fever
Tick-borne
Organism: B. duttonii
Vector: Ornithodoros soft tick
Same symptoms as above
Treatment: Doxycycline
Staphylococcus aureus
Toxic shock syndrome
Enterotoxins B and C
TSST-1 in menstrual cases
Scarlatiniform eruption and desquamation with sepsis
Staph scalded skin syndrome
Exfoliative toxin A and B
Bind to Desmoglein-1 (a transmembrane adhesion molecule in the epidermis)
At risk populations: Kids and renal disease patients
Nikolsky sign + in non-lesional skin
Culture of bullae useless no organism present (toxin mediated)
Bullous impetigo
Exfoliative toxin A and B
Others: Sycosis barbae, Botryomycosis, Acute paronychia, Felon, Endocarditis
Strep spp.
Group A Strep
Blistering distal dactylitis
Dorsal fingers and toes
Tense blisters
Perianal strep
Bright red and well-demarcated
Scarlet fever
Erythrogenic toxin A, B, and C
Strep throat
Exudative pharyngitis and strawberry tongue
Sandpaper rash starts on head and neck
Pastias lines
Linear petechiae in axillae and antecubital fossae
Purpura fulminans
Hemorrhagic infarction from DIC
Geographic areas of purpura
Others: Non-bullous impetigo, erysipelas, cellulitis (see Figure), necrotizing fasciitis,
Strep toxic-shock-like syndrome, endocarditis
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Figure 6.17: Cellulitis
Meningococcemia
N. meningitides
Gram negative diplococcus
Those at risk: Children and those with complement deficiencies (C3-5)
Petechiae may be 1st sign
IV PCN or ceftriaxone
Rhinoscleroma
Klebsiella pneumoniae rhinoscleromatis
Plaques on external nares
Mikulicz cells on path
Tx: Cipro
Mycobacterial
Leprosy
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Table 6.3: Leprosy
TT BT BB BL LL
TH1 cytokine TH2 cytokine
profile: profile IL-4, IL-10
IFN-, IL-2, IL-12 Multibacillary
Paucibacillary Lepromin test-
Lepromin test+
3 lesions 310 lesions that Many lesions Lesions too Generalized and
are smaller than distributed numerous to symmetrical
TT lesions asymmetrically count; smaller distribution
predominate
Anesthetic Similar to TT Less anesthesia Minimal or no No loss of
and anhidrotic than TT sensory defects sensation or
sweating
lesions
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6.6 VIRAL INFECTIONS
DNA Viruses
CMV
Infection during 1st and 2nd trimester - highest risk for permanent abnormalities
Small for gestational age, microcephaly, retinitis, colobomas, intracranial
calcifications
#1 infectious cause of deafness and mental retardation in US
Most common congenital viral infection
Part of TORCH syndrome with blueberry muffin baby purpura
EBV
Infects B lymphocytes
Infectious mononucleosis
Figure 6.19: Mononucleosis
Figure 6.21: Herpes simplex 1
Primary gingivostomatitis
Seen in children and young adults following primary HSV infection (usually HSV-1);
occurs in only 1% of primary HSV infections of the lips or face
Presents with fever, sore throat, and painful vesicles/erosions on the tongue, palate,
buccal, and gingival mucosa
Erosions are covered with characteristic gray membrane
Primary genital herpes
Most cases caused by primary infection with HSV-2
Multiple painful erosions, often bilateral, on the ano-genital mucosa
Painful inguinal lymphadenopathy
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Minority of cases may have concomitant aseptic meningitis with fever, nuchal rigidity,
headache, photophobia
Dysuria and vaginal/urethral discharge may be present
Severity of symptoms peaks at days 8 to 10
Herpes gladiatorum
Affects wrestlers and rugby players
Most common locations: Face, lateral neck, medial arm
Neonatal herpes simplex
Majority of cases acquired during delivery as neonate passes through infected vaginal
canal
Clinical spectrum ranges from localized skin lesions to multi-systemic infection with
encephalitis, hepatitis, pneumonia, and coagulopathy
Herpes simplex treatment
Acyclovir
Guanosine analogue
Inhibits viral DNA polymerase after being phosphorylated by viral thymidine kinase
(TK), and 2 additional viral kinases
Famciclovir
Prodrug of penciclovir; Increased bioavailability and longer half-life
Also dependent on viral TK for activity
Valacyclovir
Viral TK-dependent; same mechanism of action as acyclovir
Thrombotic thrombocytopenic purpura reported using high doses in
immunosuppressed patients
Acyclovir-resistant HSV* treatment
*Most commonly due to TK-deficient strains of HSV
Foscarnet directly inhibits viral DNA polymerase (without requiring
phosphorylation by TK)
Cidofovir inhibits viral DNA polymerase in a TK-independent fashion
Milkers Nodule
Paravaccinia virus
Cows
1 cm nodule on arm or finger
No treatment necessary
Molluscum
Poxvirus
Clinical: Umbilicated white papules
Orf
Parapoxvirus
Sheep and goats
Dorsal index finger
6 stages: Papular, target, acute, regenerative, papillomatous, regressive
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Parvovirus B19 (the only ssDNA virus)
Smallpox
Poxvirus - Variola
Incubation is an average of 12 days
Virus replicates during this time
Not contagious
Prodrome -3 days of fever, malaise, HA, back pain, and vomiting
Rare swimming trunk petechiae is pathognomonic
Generalized centrifugal eruption
Over 2 weeks
Lesions in same stage
Umbilicated lesion
Head and extremities > trunk
Most infectious during the 1st 7 to 10 days following rash onset
Contagious until scabs fall off
RNA Viruses
Measles
Paramyxovirus
Clinical
Prodrome fever, cough, coryza, and conjunctivitis
Enanthem: Koplik spots
Exanthem: Morbilliform eruption starting on face
Hand-foot-and-mouth disease
Coxsackievirus A16 or enterovirus 71
Oraloral and fecaloral mode of transmission
Togavirus Rubella
Rhabdovirus Rabies
Retrovirus HIV, HTLV
Picornavirus Enterovirus: Coxsackie virus
(Hand-foot-and-mouth disease)
6.7 STDS
Primary Syphilis Chancroid Granuloma Inguinale Lymphogranuloma
Venereum
Causative T. pallidum H. ducreyi Calymmatobacterium Chlamydia
Organism granulomatis trachomatis L1, L2, L3
Characteristic Painless chancre Soft, painful/tender Primary lesion: Papule, Painless, soft
Clinical with ham-col- chancre with subcutaneous nodule erosion that heals
Features ored base and ragged edges (pseudobubo), or spontaneously
sharply defined, ulcer
indurated border School of fish on Secondary inguinal
Gram or Giemsa 4 clinical forms: adenopathy with fluc-
Chancre has stain Ulcerovegetative tuant, tender nodes
cartilage-like (most common), above and below
consistency and nodular, hypertrophic, Pouparts ligament
exudes clear fluid and cicatricial groove sign (can be
bilateral)
Bilateral Donnovan bodies
(safety-pin shaped Serologic diagnosis
intracytoplasmic inclu- by complement fixa-
sions in macrophages) tion test
seen on microscopy
Treatment Penicillin Azithromycin TMP-SMX Doxycycline
Ceftriaxone Doxycycline
Ciprofloxacin Erythromycin
Erythromycin Ciprofloxacin
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Chancroid
H. ducreyi
Gram negative rod - School of fish appearance
Painful ulcer
Tx: Azithromycin
Lymphogranuloma Venereum
Chlamydia trachomatis L1, 2, 3
Painless erosion that heals spontaneously
Fluctuant inguinal lymphadenopathy
Groove sign
Dx: Complement fixation test
Tx: Doxycycline x 3 weeks
Gonococcemia
N. gonorrhoeae
Hemorrhagic pustules on red bases
Arthralgias
May have C5-C9 deficiency
Treatment with ceftriaxone
Syphilis
T. pallidum
Primary
Figure 6.25: Secondary syphilis
4 to 12 weeks
Skin and mucous membranes - pityriasis rosea-like
Ham-colored macules on palms
Condyloma lata
Split papules - oral commissures
Moth-eaten alopecia
Tertiary
Cardiovascular and neurological sequelae in some
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Congenital ~ Secondary syphilis
Early <2 years
SGA
Saw-tooth metaphysis
Snuffles
Rhinitis
Rhagades
Parrots lines
Late >2 years (think bony abnormalities)
Mulberry molars
High-arched palate
Hutchinsons teeth (wide spaces and peg-shaped)
Saddle nose
Saber shins
Cluttons joints (non-tender edema of knees)
Higoumenakis sign (UL medial clavicle enlargement)
Hutchinsons triad Hutchinsons teeth deafness interstitial keratitis
Serology
Nontreponemal tests
VDRL: 4 to 5 weeks after inoculation
May revert in latency
RPR: Similar to VDRL
Treponemal
FTA-ABS: 3 weeks after inoculation
Remains + after treatment
Most sensitive test
MHA-TP
Similar to FTA-ABS, but less sensitive
Treatment
Primary and secondary without end-organ damage
Benzathine PCN G 2.4 mil U IM X 1 doxycycline 100 mg BID x 2 week if PCN
allergic
Tertiary without neuro diseasebenzathine PCN G 2.4 mil U IM weekly X 3
Jarisch-Herxheimer reaction
Fever, HA, LAD, skin lesions, myalgias, WBCs
Caused by TNF-alpha and other cytokines being released when spirochetes are
phagocytosed
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Trypanosomiasis
African
T. brucei gambiense in West Africa
T. brucei rhodesiense in East Africa
Vector: Tsetse fly/Glossina
Winterbottoms sign
Posterior cervical LAD
American (Chagas disease)
T. cruzi
Vector: Reduviid beetle aka assassin bug
Clinical: Romana sign
Eyelid edema and conjunctivitis at site of inoculation
Chronic: Cardiac infiltration
GI: Toxic megacolon
Filariasis
Lymphedema and elephantiasis
Brugia malayi and timori; Wuchereia bancrofti
Vectors: Mosquitos - Aedes, anopheles, culex, and mansonia
Dracunculiasis
D. medinensis Cyclops copepods
Worms under the skin
Tx: Slowly remove parasite
Loa Loa
Mango or deer flies
Vector: Chrysops
Clinical: Calabar swellings = dead filarial in microvasculature causing nonpitting edema of
cheeks
Eye worm
Treatment with diethylcarbamazine
Onchocerciasis
O. volvulus
Vector: Black flies aka Simulium
Pruritic papules
Chronic: Leopard skin, onchocercoma (nodules of organism)
River Blindness: # 1 cause of acquired blindness worldwide
Treatment with ivermectin
Mazzotti reaction: Symptom complex seen in patients after undergoing treatment of
onchocerciasis with the medication diethylcarbamazine (DEC)
Life-threatening, and are characterized by fever, urticaria, swollen and tender lymph
nodes, tachycardia, hypotension, arthralgias, oedema, and abdominal pain
Strongyloidiasis
S. stercoralis
Larvae penetrates through skin or mucous membrane
Cercarial Dermatitis
Swimmers itch
Penetrate skin while swimming in northern US or Canadian waters
Pruritic papules on non-covered skin
Seabathers Eruption
Edwardsiella lineatasea anemone
Linuche unguiculatathimble jellyfish
Eruption is on covered skin - pressure from clothing releases toxin
Mites
Scabies
Extremely pruritic burrows/papules in skin folds and acral webs, transmitted through
physical contact, particularly in crowded jails, college housing, and nursing homes
Can have atypical presentation in infants bullous, involve the genitals
Testing = scabies prep skin scraping showing scabies mites, eggs, or feces
Treatment = permethrin 5% cream repeated in 7 days, severe cases oral ivermectin,
linens/clothing washed in hot water
Ticks
Lyme disease
Transmitted by deer ticks, most common vector-borne disease in the US
Clinical
Early localized = erythema migrans (expanding targetoid rash) 1 to 2 weeks after bite
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Early disseminated = within 6 months of bite, disseminated rash, polyarthritis, Bells
palsy
Late/chronic = months to years later, neurologic and rheumatologic symptoms
Serologic testing = unhelpful acutely
Treatment = single dosage doxycycline if within 72 hours of confirmed bite to prevent
Lyme, otherwise doxycycline (amoxicillin if allergic) x 10 to 14 days
Rocky Mountain Spotted Fever
Transmitted by dog/wood ticks, most common fatal tick-borne illness in US
Clinical
Acral petechial rash spreading centrally within a week of bite, fever, and headache
may precede
Serologic testing = unreliable
Treatment: Doxycycline x 7 to 14 days (chloramphenicol if severely allergic),
delayed treatment leads to hospitalization, death in up to 5% of cases (vascular
damage, shock)
Spiders
Black widow
Lice
Transmitted through physical contact, extreme pruritus and localized lymphadenopathy
Head lice Most common louse, laying eggs at the base of hair (nits)
Pubic (crab) lice Different body type than other lice (crab-like); macula cerulea =
bluish macules in groin (characteristic)
Human body lice Located on clothing, not body. Vector for trench fever, epidemic
typhus, and relapsing fever
Treatment: Comb removal of lice/nits, topical permethrin or malathion (flammable) all
repeated twice within a week, discarding or laundering clothing/linens in hot water
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Squamous cell carcinoma
Keratotic papules, nodules, patches/plaques; higher risk of invasion if scalp/face
Brown to black (but can be any color) macule, papule, or nodule with differing clinical
features than patients other skin lesions/nevi, local and distant metastatic potential,
can occur anywhere on skin/mucosa
A(symmetry), B(order), C(olor), D(iameter), E(volving)
Complete excisional biopsy required for tumor depth (Breslow, determines prognosis)
Treatment: Wide local excision, possible sentinel lymph node sampling and body
imaging, adjuvant therapies inconsistently effective for metastatic disease, but targeted
biologic agents evolving
Risk factors for the development of MM include sun exposure with tendency to freckle
and sunburn, fair skin complexion, light colored eyes and hair, presence of large
number of nevi, as well as family and personal history of melanoma
The most important mutated gene associated with a predisposition to develop MM is
the CDKN2A (located on chromosome 9p21)
Skin tag (acrochordon): Fibrous growth of skin, often multiple tan pedunculated papules
particularly in areas of friction, with genetic and metabolic syndrome link
Corn (clavus): Keratotic firm inverted growth, often on plantar surface, due to friction or
pressure
Epidermoid and pilar cyst: Epidermoid cysts are nodules often with central punctum often
on trunk; pilar cysts are dermal nodules with no punctum commonly on the scalp; all have
potential for growth and rupture
Neurofibroma: Pink fleshy papules, sometimes tender; multiple in neurofibromatosis
syndromes
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Pyogenic granuloma: Enlarging, easily traumatized red friable papules, can favor the face
and digits, more common in children, pregnancy, and with certain HIV drugs; self-resolving
but often removed because of tenderness (particularly periungual), and bleeding
Venous lake: Dark red to purple papule made of blood vessels, commonly on the lip
Cherry angioma: Red papules favoring the trunk, typically increasing in number over time
Dermatofibroma: Firm dusky pink to brown papules with dimple sign on exam, commonly
on legs of females, sometimes precipitated by trauma
Hypertrophic scars and keloids: Hypertrophic scars are firm pink shiny plaques within the
confines of an established scar; keloids grow bulbously beyond the original scar and can
often occur at piercing sites
Sebaceous hyperplasia: Pink to orange doughnut-shaped small papules on the face of
adults
Digital mucous cyst: Tense shiny nodule near DIP, representing joint fluid leakage because
of osteoarthritis/injury; drainage of the viscous fluid can temporarily relieve pressure but
ortho/hand surgeon intervention is often needed to ultimately prevent recurrence
6.12 PRURITUS
Overview
Pruritus
Transmitted via unmyelinated C and A delta fibers
Skin and cornea only tissues that itch
Histamine #1 mediator
Others include papain, trypsin, serotonin, bradykinin, kallidin, kallikrein, substance P, VIP
Prostaglandins exaggerate existing itch
Opiates have both central and peripheral itch-producing action
6.13 URTICARIA/ANGIOEDEMA
Urticaria
Individual lesions called wheals; may have pale centers (not dusky, so not targetoid)
By definition lesions should last <24-hours
Ask the patient: Do they move around?
Note: Chronic urticaria defined as 6 weeks
Urticaria may be a disease spectrum with depth of swelling causing urticaria, angioedema
(deeper in dermis/subq), or both
Type I hypersensitivity
Often caused by: Idiopathic, drug, food, infection (GAS, viral, parasites)
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Primary effector cell = mast cell
Pre-formed mediators = histamine, heparin, tryptase, chymase
Newly formed mediators = prostaglandins, leukotrienes, PAF
Types of urticria:
Physical urticaria
Dermatographism
Pressure urticaria
Cold urticaria
Solar urticaria
Angioedema
Urticarial Vasculitis
Clinically indistinguishable from urticaria, but last >24-hours
Hypocomplementemic urticarial vasculitis syndrome
Defined by low serum complement levels plus presence of anti-C1q precipitin (in 100%),
decrease in C1 activity
Lupus Erythematosus
Acute
Types: Malar erythema butterfly rash, diffuse erythemas, bullous
These acute findings are usually associated with SLE, but there is overlap
Systemic lupus erythematosus (SLE)
Know the 11 criteria, 4/11 = SLE;
4 skin findings: Malar, discoid, oral ulcers, photosensitivity
2 antibodies: ANA (99% sensitive) and anti-Smith (or anti-dsDNA) (specific)
5 systems: Heme (hemolytic anemia, leukopenia, or thrombocytopenia), renal
(proteinuria), neuro (seizures or psychosis), rheum (arthritis), and cards/Pulm
(serositis)
Note- ANA patterns: Homogeneous (anti-histone), peripheral (anti-dsDNA, SLE
with renal dz), speckled, nucleolar (ribosomal RNA, scleroderma), centromeric
(Only centromeric is specific: CREST)
Lupus band test = DIF shows DEJ IgG deposits in normal, non-sun-exposed skin of
SLE
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Drug-induced lupus
Resolves in days to months
Most common: Hydralazine (5%), procainamide (15 to 25% of patients taking the
drug), quinidine
Has been reported with minocycline
Anti-histone Ab in 95% - this is positive in 50% of SLE
Subacute
Types: Annular (and polycyclic), urticarial, papulosquamous
Subacute cutaneous lupus (SCLE)
Strong anti-Ro association, tend to be ANA positive
Can develop Sjgren; half may meet criteria for lupus
Drug-induced by HCTZ, terbinafine (Lamisil) > Ca channel blockers, NSAIDs,
griseofulvin, antihistamines
Neonatal lupus erythematosus
Risk of 3rd degree heart block (15 to 30%)
Check for Ro (most common in 95%), La, anti-U1-RNP
Mother usually asymptomatic, usually Ro positive (1% risk)
25% risk of next child developing
Chronic
Types: Discoid, panniculitis, hypertrophic/lichenoid
Discoid lupus (DLE)
Dermatomyositis
Clinical signs
Gottrons papules = lichenoid papules over MCPs, DIPs, PIPs
Gottrons sign = pink/red/purple atrophic or scaling eruption over knuckles, knees,
elbows
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Non-specific: Heliotrope rash (ddx contact dermatitis, trichinosis), poikiloderma,
mechanics hands, malar erythema
Remember: Amyopathic dermatomyositis (atypical case without muscle
involvement)
Frayed cuticular changes in DM = Samitz sign
Important: r/o underlying malignancy (especially likely if age >50)
R/o paraneoplastic syndrome, in women: Ovarian/breast cancer; Men: GI, respiratory
cancer
Antibodies: Anti-Jo-1 (predicts lung involvement), anti-Mi-2 (predicts benign course, less
lung involvement), anti-Ku (DM/scleroderma overlap)
Can see elevated CK and aldolase
Scleroderma
Localized (morphea)
Sjgren
Keratoconjunctivitis sicca
Xerostomia
Rheumatoid arthritis
More than 90% women
Vasculitis palpable purpura
Patients develop lymphoreticular malignancy such as NHL
Anti-Ro and La, anti-a-fodrin
Rheumatoid Arthritis
Can be associated with vasculitis
Can be underlying cause of epidermolysis bullosa acquisita
Rheumatoid nodule
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Relapsing Polychondritis
On ears, recognized by only affecting cartilage
Can also affect nose, larynx
Can see Ab to type II collagen (but found in <50%) - Car-2-lage
Concern regarding tracheal involvement
Can be associated with MAGIC syndrome (Mouth And Genital ulcers and Inflamed Cartilage
= relapsing polychondritis + Behets)
Pemphigus foliaceus - superficial, rarely appreciate intact vesicles. Bran flake-like crust
Pemphigus vulgaris (mucocutaneous)
Pemphigus erythematosus (lupus overlap)
Paraneoplastic (associated with NHL, CLL, thymoma, sarcoma, Castleman)
Drug induced
Thiol-containing drugs: Captopril, penicillamine, thioproline
Drugs with disulfide bonds: Gold, pyritinol
Drugs that have the potential to release sulfur moieties: Penicillins, piroxicam,
cephalosporins
Pyrazoline derivatives and enalapril (possibly secondary to an amide group),
indomethacin, rifampin
Subepidermal Blisters
Clinical manifestations and diagnosis: Tense/ruptured bullae, diagnosed by skin biopsy/
immunofluorescence
Bullous pemphigoid occurs in elderly and can look like urticaria (hives)
Dermatitis herpetiformis (DH) extremely pruritic on extremities, often with concurrent
celiac disease
Linear IgA has necklace-like linked bullae, can be caused by vancomycin
Bullous lupus form of acute lupus
Treatment: Systemic and topical steroids, dapsone, and gluten-free diet very helpful in DH
6.16 ALOPECIA
Non-scarring Alopecia
Alopecia areata
Genetics
High frequency of family history, especially in patients with early onset (37%)
Twin concordance = 55% (identical twins)
Immunologic factors
Major associations: Vitiligo and thyroid disease (10%), with increased prevalence of
antithyroid antibodies and thyroid microsomal antibodies in AA
Other autoimmune diseases shown to be associated: Pernicious anemia, diabetes,
LE, myasthenia gravis, RA, polymyalgia rheumatica, ulcerative colitis
Emotional stress
May be precipitating factor in some cases
Clinical features
Pull test may be positive at margins, indicating early disease
Usually asymptomatic, but some patients perceive pruritus, tenderness, burning, or
pain preceding hair loss
PATTERNS: Patchy (most common); reticulated; ophiasis (parietal/temporal/occipital);
ophiasis inversus (sisapho bandlike pattern in fronto parietotemporal scalp)
Areata partial loss of scalp hair
Totalis total loss of scalp hair
Universalis 100% loss on scalp, eyebrows, eyelashes, and rest of body
Initial regrowth is white, followed by repigmentation
Nail dystrophy (10 to 66%), seen in one, some, or all nails, preceding, coinciding, or
occurring after hair disease
Pitting with irregular pattern or in organized rows
Trachyonychia: Longitudinal striations resulting in sandpaper appearance
Red-spotted lunula
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Treatment: Intralesional steroids, topical steroid, systemic steroid, PUVA, topical
immunotherapy (e.g., anthralin, diphenylcyclopropenone, squaric acid dibutyl ester),
minoxidil
Triangular Alopecia
Congenital or childhood
Complete absence of hair or vellus hairs in triangular pattern in the temporal area,
frequently bilateral
Androgenetic Alopecia
AD, polygenetic with variable penetrance
Progressive miniaturization of hair, increased telogen hairs
Males bitemporal, vertex
Females preserved anterior hair line, Christmas-tree pattern with widened hair part at
vertex
Type II 5-alpha reductase activity in dermal papilla and outer root sheath
TX: Minoxidil, finasteride, oral contraceptive pills, spironolactone, flutamide, cyproterone
acetate, transplant
Trichotillomania
Compulsive hair pulling, irregular broken hairs within a geometric localized area
TX: Chlorimipramine, SSRI
Syphilis
Secondary syphilis, 3 to 7% occurrence rate
Moth-eaten non-scarring with indistinct margins or diffuse alopecia
Telogen Effluvium
Early and excessive loss of club hairs from the normal resting follicles in the scalp
Physical stress such as: Surgery, anemia, traction or systemic illness - generally 3 months
prior to onset
Psychological stress
Endocrine causes such as: Hypo or hyperthyroidism or peri-/postmenopausal states
Nutritional deficiencies: Biotin, iron, protein (kwashiorkor), zinc, essential fatty acid or calorie
deficiency (marasmus or starvation diets)
Hypervitaminosis A
Drugs implicated:
Amphetamines
Aminosalicylic acid
Angiotensin-converting enzyme inhibitors
Anticoagulants
b-blockers
Bromocriptine
Anagen Effluvium
Frequently seen following administration of cancer chemotherapeutic agents
Stimulus induces the abrupt cessation of mitotic activity in rapidly dividing hair matrix
cells; hair shaft thins and then breaks at skin surface
Occurs within days to weeks of the stimulus
Entirely reversible with cessation of drug therapy
Causes
Antimetabolites
Alkylating agents
Mitotic inhibitors
Examples: Doxorubicin, the nitrosoureas, and cyclophosphamide
Alopecia (Scarring)
Pseudopelade of Brocq
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Alopecia in small patches foot prints in the snow to large patches
Controversy regarding distinct entity versus end-stage of various scarring alopecias
Follicular degeneration syndrome
Hair loss mostly on vertex in black females +/ history of chemical relaxers
Previously known as hot comb alopecia but can occur without use of hot combs
Lichen planopilaris
Other Alopecia
Meralgia paresthetica
May have alopecia of the anesthetic area of the outer thigh
Hypothyroidism
Hair coarse, dry, brittle, and sparse
Telogen hairs 3x more prevalent
Hyperthyroidism
Hair becomes extremely fine and sparse
Alopecia neoplastica
Hair loss from metastatic tumors
Usually breast carcinoma
Cardiovascular Disease
Hypertension, venous stasis stasis dermatitis stasis dermatitis on legs, resembles cellulitis
but shows bilateral involvement with background stasis changes; often confused for
bilateral cellulitis
General hyperlipidemia various types of xanthomas
Hypertriglyceridemia eruptive xanthomas
Metabolic syndrome acanthosis nigricans, skin tags (*patients with psoriasis carry a higher
risk of metabolic syndrome then the general population
Superior vena cava syndrome facial edema and dilated veins on skin superior to the heart
on the upper chest
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Endocrine Disease
Hypothyroidism
Cool, dry, pale skin, xerosis, hypohidrosis, yellowish hue secondary to carotenemia
Generalized myxedema swollen waxy appearance to skin and lips, broad nose,
macroglossia
Dry, brittle, coarse, straw-like hair
Hyperthryoidism
Thyroid acropachy clubbing of fingers associated with soft tissue swelling and
periosteal new bone formation
Localized or generalized hypertrichosis, wispy fine hair
Thyroid dermopathy (pretibial myxedema) bilaterally symmetric, non-pitting
yellowish-brown to red waxy papules, nodules, and plaques most commonly on lower
extremities (but can occur on any area of skin)
Diabetes mellitus
Acanthosis nigricans, diabetic limited joint mobility (cheiroarthropathy), necrobiosis
lipoidica (NL), scleredema, diabetic bullae (bullosis diabeticorum), diabetic dermopathy
(shin spots or pigmented pretibial papules), eruptive xanthomas, granuloma annulare
(generalized and perforating forms), skin tags, perforating dermatoses
Can also be associated with other autoimmune skin conditions like vitiligo
Rheumatologic Disease
Lupus
Photosensitive eruptions butterfly rash (acute systemic lupus), discoid (scarring
especially on face and inside ears), annular (subacute cutaneous lupus)
Vasculitis palpable purpura
Alopecia, often scarring
Dermatomyositis
Photosensitive eruption, heliotrope, Gottrons papules, ragged cuticles
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Gastrointestinal Disease
Inflammatory bowel disease
Erythema nodosum; ulcerative colitis (UC) > Crohns
Cutaneous/metastatic Crohns vegetative ulcerating plaque with histologic features of
Crohns (granulomatous)
Pyoderma gangrenosum ulceration with underminable rolled dusky borders; +
pathergy (do not debride); UC > Crohns
Gingival hyperplasia and cobblestoning (Crohns)
Pyoderma vegetans (UC)
Vasculitis/Polyarteritis nodosa (Crohns)
Celiac disease
Dermatitis herpetiformis very pruritic, especially on elbows
Over 90% of people with the rash have gluten-sensitive enteropathy
15 to 25% celiac patients develop dermatitis herpetiformis
Skin disease responds to gluten-free diet (and dapsone)
Hepatitis C
Porphyria cutanea tarda sun-exposed papules, bullae, tiny cysts and scars, werewolf-
like hair growth on face, 24-hour urine porphyrins is test of choice
Lichen planus purple polygonal pruritic papules, classic area is wrists but can occur
anywhere on skin, Wickham striae (can see in mouth), oral erosions (most commonly in
setting of Hep C)
Necrolytic acral erythema psoriasiform plaques on dorsa of feet
Figure 6.53: Porphyria cutanea tarda
Pulmonary Disease
Sarcoidosis
Lupus pernio plum-colored nodules especially on nose, cheeks, and ears
Erythema nodosum
Wegener granulomatosis
Palpable purpura on legs, skin ulcers, erythema nodosum
Oral ulcers and gingival hyperplasia (strawberry-like)
Pulmonary hypertension
Clubbing
Renal Disease
Renal failure
Pruritus best treated with light therapy
Nephrogenic systemic fibrosis (NSF) thickening of skin (excluding face), caused by
renal insufficiency paired with exposure to MRA with gadolinium contrast
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Calciphylaxis purple necrotic plaques (the more proximal, the more deadly), caused
by calcifications of vessels and thrombosis, often results in severe wounds, infection,
and death
Perforating dermatoses crusted papules on skin (especially arms) representing
extruding material from the body
Neurologic Disease
Alzheimers, Parkinsons, stroke seborrheic dermatitis, increased risk of melanoma
Neurocutaneous syndromes
Neurofibromatosis neurofibromas, skin freckling (axilla), caf au lait macules, Lisch
nodules in iris, scoliosis, seizures
Tuberous sclerosis facial papules (adenoma sebaceum), ash leaf macules
(hypopigmented), Shagreen patch (thick leathery truncal plaque), intracranial tubers
causing various neurologic symptoms
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Erythroderma
Causes: Psoriasis, atopic dermatitis, drug eruption, cutaneous lymphoma, paraneoplastic,
idiopathic
Clinical manifestations: Diffusely erythematous scaly skin, sometimes with ectropion
Diagnosis and management: Clinical history and skin biopsy; treatment = etiology-based,
often topical and systemic steroids and antihistamines, IV fluids
Transplant patients: Skin cancer (highest risk is for squamous cell carcinoma 36 fold),
graft-versus host disease (GVHD), disseminated infections
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TB and atypical mycobacteria (esp. MAC)
Cryptococcosis
Toxoplasmosis
Reactive arthritis (psoriasiform)
Diffuse verruca vulgaris/ condyloma acuminata
Pruritus
Can also see: Acquired ichthyosis, drug reactions, and psoriasis
Types of Cutaneous TB
From external exposure:
Primary inoculation
Tuberculosis verrucosa ctis
Tuberculosis cutis orificialis (orificial TB)
From hematogenous spread:
Lupus vulgaris
Miliary tuberculosis
From direct extension:
Scrofuloderma
Reactive eruptions to TB (tuberculid eruptions):
Papulonecrotic tuberculid
Lichen scrofulosorum
Erythema induratum
Causes of Urticaria
Mnemonic = Mr. Hives still seeks drugs and graft
Viral (MR. HIVES) = Measles, Rubella, Hepatitis, Infectious mono (including EBV, CMV, HIV),
Viral other, E. infectiosum/Subitum
Dermatome Review
Thumb = C6
Nipple = T4
Umbilicus = T10
Top of feet = L5
Bottom of feet = S1
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REFERENCES & SUGGESTED READINGS
1. Freedberg IM, Eisen AZ, Wolff K, et al., eds. Fitzpatricks dermatology in general medicine. 5th ed.
New York: McGraw-Hill, Health Professions Division, 1999.
2. Ingham E et al. Proinflammatory levels of interleukin-1 alpha-like bioactivity are present in the major-
ity of open comedones in acne vulgaris. J Invest Dermatol. 1992; 98: 895-901.
3. Kim J et al. Activation of toll-like receptor 2 in acne triggers inflammatory cytokine responses. J
Immunol. 2002; 169:1535-1541.
4. Andrews diseases of the skin, 9th ed. Philadelphia: WB Saunders, 2000.
5. Goldstein SM and Wintroub BU, Adverse cutaneous reactions to medications. Baltimore: Williams
and Wilkins, 1996; 55-57.
6. Wolverton, SE. Comprehensive dermatologic drug therapy. Philadelphia: WB Saunders, 2001.
7. Wolverton, SE. Comprehensive dermatologic drug therapy. Philadelphia: WB Saunders, 2001.
8. Elder D et al. Levers histopathology of the skin, 8th ed. Philadelphia: Lipincott Raven, 2001; 295.
9. Webster G Fetal. Suppression of polymorphonuclear leukocytechemoactic factor production in
Propionibacterium acnes by subminimal inhibitory concentrations of tetracycline, ampicillin, minocy-
cline and erythromycin. Antimicrob agents chemother. 1982; 21:770-772.
10. Wolverton,SE.Comprehensivedermatologicdrugtherapy.Philadelphia:WBSaunders,2001.
11. Dermatology in General Medicine, 5th Edition. New York, McGraw-Hill,1999.
12. Andrews Diseases of the Skin, 9th Edition. Philadelphia:WB Saunders,2000.
13. http://www.cdc.gov/lyme/
14. http://www.cdc.gov/rmsf/
15. http://www.cdc.gov/parasites/lice/
16. Goldsmith L, et al. Fitzpatricks Dermatology in General Medicine 8e, New York: McGraw Hill, 2012.
17. Rigopoulos D, et al. Skin signs of systemic diseases. Clin Dermatol. 2011; 29(5): 531-40.
18. Thiers BH, et al. Cutaneous manifestations of internal malignancy. CA Cancer J Clin. 2009; 59(2):
73-98.
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NOTES
Matthew J. Mandel, MD
c o n t e n t s
7.2 IMAGING . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
7.3 STROKE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
7.5 EPILEPSY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
7.7 DEMENTIA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
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7.1 Neurologic Examination
The neurologic examination has 7 basic parts: 1. Mental status 2. Cranial nerves 3. Motor
4. Sensory 5. Reflexes 6. Coordination 7. Gait
The general purpose of performing a neurologic exam is to localize the lesion; can be
thought of as a "poor mans MRI"
Mental Status
Abnormalities on mental status testing indicate a problem in higher cortical areas (ex.:
decreased attention testing localizes to the frontal lobe)
Assess with a mini-mental status examination including: Alertness, orientation,
concentration, memory, calculations, abstract reasoning, fund of knowledge
Cranial Nerves
Abnormalities on cranial nerve testing indicate a problem in the brainstem (composed of
the midbrain, pons, and medulla)
II = optic n; III = oculomotor n; IV = trochlear n; V = trigeminal n; VI = abducens n; VII = facial
n; VIII = vestibulochoclear n; IX = glossopharyngeal n; X = vagus n; XI = accessory n;
XII = hypoglossal n
Motor
Abnormalities on motor testing could indicate a problem anywhere from the neurons that
form the corticospinal tracts to the neuromuscular junction to the muscle itself
Strength is graded on a 5-point (MRC) scale: 5/5 = normal; 3/5 = able to move vs. gravity
but not vs. resistance; 1/5 = twitch
Sensory
Abnormalities on sensory testing could indicate a problem anywhere from the sensory
receptors to the higher cortical areas where sensory input is processed
Assessments of light touch, pin, vibration, and proprioception should be performed
Reflexes
Abnormalities of reflex testing indicate problems at various spinal cord levels
Coordination
Abnormalities on coordination testing indicate problems with the cerebellum
Gait
Gait is multifactorial; does not necessarily localize to any 1 area of the CNS; however,
certain gaits are characteristic of specific neurologic conditions (ex.: shuffling gait think
Parkinson's disease)
MRI Scans
Depending on protocol, can take up to 45 minutes to perform study
Different sequences appropriate for different conditions
T1 = anatomy
T2 FLAIR = multiple sclerosis
Diffusion weighted imaging (DWI) = stroke
7.3 Stroke
Classification (By Pathophysiology)
Ischemic - due to thromboembolic occlusion of blood flow
Hemorrhagic extravasation of blood into the brain parenchyma (ICH) or spaces (SAH)
Ischemic Stroke
Acute Ischemic Stroke
Definition
Rapid onset of focal neurologic deficits in a neuroanatomical distribution (i.e., MCA
syndrome, ACA syndrome)
If the neurologic deficits last <24-hours the condition is technically called a transient
ischemic attack (TIA)
Evaluation and Treatment
If <3 hours since the onset of symptoms, rapid evaluation and diagnosis is critical as
administration of tPA can lead to revascularization
Brief history from patient or family members present in the ER; most important to
establish time of onset
Performance of NIH Stroke Scale
Non-contrast head CT to make sure no hemorrhage
Coagulopathy labs PT/INR, platelets
Rapid evaluation and diagnosis is critical as administration of tPA in <3 hours from
onset can lead to revascularization
If If >3 to 4 1/2 hrs since the onset of symptoms, tPA is contraindicated as the risk of
secondary hemorrhage outweighs the benefits of potential revascularization
Orders: Admit, ASA 325 mg po, statin, hold antihypertensive meds, MRI brain/
MRA head and neck, telemetry, UA/Umicro/Utox, CXR, EKG, TTE, carotid dopplers,
swallow evaluation, HbA1c, fasting lipids, DVT ppx, PPI, nicotine patch, maintain
normoglycemia, PT/OT
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Secondary Prevention
The same vascular risk factors that put a patient at risk for myocardial infarction, put a
patient at risk for TIA and stroke. Good control of HTN, diabetes, cholesterol, and weight
will minimize the occurrence of stroke/TIA. Regular physical activity and minimization of
tobacco and ETOH consumption also minimize the occurrence of stroke/TIA
Coagulopathies and Afib can increase the chance of embolus formation and subsequent
stroke
Hemorrhagic Stroke
Intracerebral Hemorrhage (ICH)
Etiology
Most commonly due to HTN; if patients blood pressure acutely rises above their normal
chronic hypertensive level, suspect hemorrhage (acute reactive HTN)
Treatment
BP control
Prognosis
Small ICH (<30 mL) favorable outcome
Large ICH often death
Subarachnoid Hemorrhage (SAH)
Etiology
Most commonly aneurysmal (in the Circle of Willis)
Diagnosis
Classic history = sudden onset of worst headache of life (i.e., thunderclap headache)
Non-contrast head CT to reveal blood is the initial test of choice and will make the
diagnosis in 95% of cases. However, if the scan is negative, an LP must be performed to
diagnose the remaining 5% of cases
Graded clinically 1 (asymptomatic or minimal headache/slight neck stiffness) - 5 (deep
coma; decerebrate rigidity) on the Hunt & Hess Scale
Treatment
Medical: Admit to NICU, CT angiogram, amicar until intervention (started in ED), seizure
prophylaxis, SBP<140, nimodipine 30/60 mg po Q4, fluids, blood glucose control, check
UA, umicro, utox, HbA1c, TTE, serial trop and EKG, hold lovenox until 24-hours after
intervention. SCDs for DVT ppx until then
Surgical: Clipping (open procedure) or coiling (neurointerventional) of aneurysm
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Demyelinating Diseases (Other)
MS is by far the most common demyelinating disease
Other demyelinating diseases include:
NMO (neuromyelitis optica) lesions in optic nerve and spinal cord without lesions in
the brain
ADEM (acute disseminated encephalomyelitis) single episode of severe acute
demyelination; postinfectious/postvaccinal
Guillen Barre (GBS) Syndrome
7.5 Epilepsy
Seizures
Partial Seizure
Synonymous with focal; i.e., there was a well defined focus of seizure onset
Simple Partial
Preservation of consciousness
The aura (dj vu, nausea, etc.) that precedes certain epileptic events is actually a
simple partial seizure in and of itself
Complex Partial
Impairment of consciousness
The patient can display unusual behaviors (lip smacking, chewing, swallowing, etc.) while
the seizure is occurring, which can be perceived by others as mental illness or drug use.
Often preceded by an aura and followed by amnesia for the period of time immediately
preceding and immediately following the seizure event
Generalized Seizure
No defined focus of seizure onset; i.e., diffuse seizure activity
Primary Generalized
Onset of diffuse seizure activity is immediate
Absence
Typical history is a school aged child who the teacher believes is not paying attention
or is day-dreaming; EEG reveals frequent epiletiform activity (3 Hz spike and wave)
throughout the day; treatment = ethosuximide
Tonic-clonic
This is the classic Grand Mal seizure comprised of a phase where the muscles tense up
(tonic phase) followed by a phase where the muscles continuously contract and relax
(clonic phase). Often preceeded by an aura and followed by a postitcal (i.e., sleepy)
period
Although the generalized tonic clonic (GTC) seizure looks like a medical emergency (and
truly is sometimes), the majority of cases pass on their own before acute benzodiazepine
treatment can be administered
Secondary Generalized
Onset of diffuse seizure activity occurred after the spread of focal seizure activity
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Status Epilepticus
Definition and Diagnosis
Official definition is repetitive seizures occurring for at least 30 minutes without full
recovery between attacks. However, it is important to treat possible status epilepticus
fast and aggressively as the earlier treatment is initiated, the more likely it is that the
status epilepticus will break/resolve. Any seizure lasting more than a few minutes should
be regarded as status epilepticus
Status is a neurologic emergency because ongoing seizures will cause permanent
neurological damage due to neurotransmitter toxicity, failure to meet metabolic demands,
and systemic complications
Etiology
Consider subtherapeutic AED levels (in a patient with prior epilepsy) > stroke/CNS tumor
> toxic - metabolic (including hyperglycemia or hypoglycemia, low Ca/Na/Mg/P, high
BUN/Creat; medications) > ETOH > hypoxia > infection > TBI. One-third of cases are
idiopathic
Treatment
ABCs
IV access; send blood for chemistries/cbc/lfts/AED-levels; ABG, Utox
Thiamine 100 mg IV and D50 50 mL IV (unless fingerstick for glucose)
Lorazepam 4 mg IV x 1 over 2 min (may give additional 2 x 2 mg each after 5 min (total
of 8 mg) if still seizing
Load with fosphenytoin 20 mg/kg at 150 mg/min; monitor EKG/BP
Often, the above management will suffice in the acute setting. However, if seizures
persist, patients may receive: Midazolam, propofol, IV valproate, IV phenobarbital, IV
pentobarbital
Obtain cEEG as soon as possible
Secondary Parkinsonism
In addition to idiopathic Parkinson's disease, a Parkinsonian phenotype (including
bradykinesia, rigidity, tremor, and postural instability) can be secondary to a variety of
primary insults including:
Drugs - neuroleptics, antiemetics, and lithium; removal of the offending agent = 1st step
in treatment
Toxins - MPTP
Hydrocephalus
Infections - postencephalitic parkinsonism
Metabolic Wilson disease
Trauma
Vascular
Parkinsons-Plus Syndromes
Feature the classic Parkinsonian phenotype (bradykinesia, rigidity, tremor, and postural
instability) with additional features (such as dysautonomia, supranuclear gaze palsy, etc.;
specific to the particular syndrome) that distinguish them from simple idiopathic Parkinson's
disease
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Suspect these syndromes if there is a symmetrical onset of symptoms (vs. classic idiopathic
Parkinsons that often begins unilaterally)
These conditions generally respond less well to anti-Parkinsons drugs and are more rapidly
progressive than classic idiopathic Parkinson's disease
Examples of Parkinsons-Plus syndromes include:
Multiple system atrophy (MSA)
Autonomic features commonly occur
Progressive supranuclear palsy (PSP)
Difficulty with vertical (typically upward) gaze
Corticobasal degeneration
Alien hand syndrome (i.e., patient believes their limb is not theirs and cannot
control its movements) occurs in 60% of those diagnosed with CBD
7.7 Dementia
Reversible and Nonreversible Causes of Dementia
Reversible: Vitamin B12, thyroid disease, neurosyphilis, neoplasms, NPH (normal pressure
hydrocephalus), subdural hematoma
Nonreversible: Alzheimer's disease, Lewy body disease, Parkinson's disease, Pick disease,
PSP (progressive supranuclear palsy), HIV-associated dementia, CJD (Creutzfeldt-Jakob
disease), some vascular diseases
Alzheimer's Disease
Clinical Description and Diagnosis of Alzheimer's Dementia
Multiple cognitive deficits; memory impairment + impairment in at least 1 other cognitive
sphere (aphasia, apraxia, agnosia)
In addition to cognitive impairment, the patient may also have hallucinations/delusions,
mood disturbances (i.e., depression, agitation), sleep/wake cycle disturbances
In making the diagnosis, the physician must make sure the symptoms are not due to
delirium or another CNS/general medical condition
Incidence and Prevalence
Incidence = 53 new cases per year/1,000 people (age 65 to 74); 170 new cases per
year/1,000 people (age 75 to 84); 231 new cases per year/1,000 people (age >85)
Prevalence = 231 cases/1,000 people (age >65)
Risk Factors
Age some statistical models predict that everyone would develop Alzheimer's disease if
they were to live long enough
F>M
African American ancestry have twice the risk of developing AD as Caucasians
MCI (of amnestic type)
Family history
Lower level of education education is protective vs. Alzheimer's disease, possibly due to
the individual having a greater cognitive reserve
Head trauma
Apolipoprotein genotype
Progression of Alzheimer's Dementia
Gradual onset with progressive decline; average duration of illness = 10 years from onset
to death
Pathology
Early neuronal loss in medial temporal lobe
Senile plaques/amyloid deposition (= primarily AB-42 peptide)
Neurofibrillary tangles made of tau protein and ubiquitin
Treatment
Cholinesterase inhibitors (tacrine, donepezil, rivastigmine, galantamine)
Used for mild to moderate AD (except donepezil which is also approved for severe AD)
NMDA receptor antagonist (memantine) approved for moderate to severe AD
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Lewy Body Dementia
Characterized by dementia and Parkinsonism progressing simultaneously (in contrast to
dementia superimposed on Parkinson's disease where the Parkinson's disease is present
long before the onset of dementia)
Classic features = fluctuating attention/alertness, visual hallucinations, Parkinsonian type
motor impairment
Lewy body = an abnormal aggregation of alpha-synuclein, neurofilament, and ubiquitin
proteins
An immunoperoxidase stain for ubiquitin turns the Lewy bodies brown
Prognosis: Slowly progressive
Vascular Dementia
= Multi-infarct dementia caused by multiple small infarcts disrupting neural networks
Risk factors = the same risk factors as for MI/CVA (i.e., HTN, smoking, diabetes, etc.)
Consider this diagnosis whenever there are focal neurologic signs in addition to dementia
Typically, there is a step-wise decline in cognitive functioning (vs. AD with its more
steadily progressive decline)
Sundowning Delirium
Is a delirium on dementia; i.e., patients with dementia (an already pathologic brain) are at
increased risk of evening agitation/confusion
Exacerbated by pain, fecal impaction, malnutrition, polypharmacy, poor sleep, infections,
unfamiliar environment
Meningiomas
= Tumors of the arachnoid cells of the meninges
Generally slow growing with symptoms caused by mass effect of tumor on neighboring
brain structures
On imaging, the tumors are extra-axial (i.e., outside the brain proper because they arise
from the meninges) and exhibit homogeneous enhancement with a dural tail (where they
are connected to the meninges)
Treatment = surgery + radiation
Metastatic
Metastatic cancer to the brain is much more common (about 10:1) than primary cancer of
the brain
Incidence increases with age
Cancers that met. brain include: Lung (both SCLC & NSCLC), breast, melanoma, and renal
cell carcinoma
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Paraneoplastic Syndromes
Paraneoplastic syndromes are syndromes associated with humoral factors excreted by
tumor cells or by an antibody response against the tumor
Typically occur in middle aged/older patients
Typically occur in lung cancer, breast cancer, ovarian cancer, and lymphoma
Anti-Ri antibody = opsoclonus-myoclonus syndrome
Anti-Yo antibody = subacute cerebellar cortical degeneration syndrome
Myopathies
As the name suggests, myopathies are primary disorders of the muscle; weakness and
cramping are common
Myopathies can be due to a variety of causes including:
Inherited (muscular dystrophy)
Inflammatory (dermatomyositis, polymyositis, and inclusion body myositis)
Toxins (related to alcohol, corticosteroids, narcotic use)
Metabolic/mitochondrial
Endocrine-related (thyroid, parathyroid, adrenal, and pituitary disorders)
Paraneoplastic
Critical illness
Metabolic Myelopathies
Vitamin B12 deficiency (occurs after nitrous oxide exposure at dentists office, get subacute
combined degeneration of posterior columns and corticospinal tracts), vitamin E deficiency,
and copper deficiency all affect the spinal cord
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Compressive Myelopathies
Metastatic cancer of breast, lung, kidney, prostate; areas affected = lumbar > thoracic >
cervical; txt = dexamethasone (10-100 mg loading dose 24 mg q6h)
Primary neoplasms of the spinal cord (meningioma, schwannoma, neurofibroma,
ependymoma)
Spondylosis (degenerative disease of vertebrae leading to compression; often C5-C6 or
C6-C7 levels)
Epidural abscess (60% of cases = staph aureus)
Trauma
Concussion
Definition: Any brief loss or alteration of consciousness 2/2 head trauma; a functional
impairment due to jarring of the brain
Grading of concussions
Grade 1 = no LOC (loss of consciousness), confusion = transient, time to symptom
resolution <15 minutes
Grade 2 = no LOC, confusion = transient, time to symptom resolution >15 minutes
Grade 3 = + LOC
Returning to play after concussion
Grade 1: May return to play if symptoms resolve in <15 minutes
Grade 2: May return to play in 1 week
Grade 3: May return to play in 1 to 2 weeks
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NOTES
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NOTES
Radu V. Saveanu, MD
Kathleen Molly McShane, MD, MPH
Luis Chaves, MD
Harvey Chitiva, MD
C o n t e n t s
8.7 SCHIZOPHRENIA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
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8.1 ANXIETY DISORDERS
Generalized Anxiety Disorder (GAD)
Excessive anxiety and worry about a number of activities, occurring more days than not, for
at least 6 months
The worry is difficult to control
At least 3 of the following 6 symptoms are present:
Restlessness or feeling keyed up
Difficulty concentrating or mind going blank
Irritability
Easily fatigued
Muscle tension
Sleep disturbance
Treatment
Medications
SSRIs and SNRIs (see Table on Common Antidepressants)
Benzodiazepineslong acting (e.g., clonazepam) is preferred over short acting (e.g.,
alprazolam) to avoid withdrawal-induced anxiety and to lower risk of abuse (see
Table on Common Anxiolytics)
Buspironea nonbenzodiazepine anxiolytic, useful if history of substance abuse
Psychotherapy
Cognitive behavioral therapy to correct cognitive distortions (e.g., catastrophizing)
and to teach relaxation techniques and desensitization
Diagnosis
Recurrent unexpected panic attacks
1 or more of the following:
Concern about having additional attacks
Worry about the implications or the consequences (e.g., losing control, having a heart
attack)
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Table 8.2: PTSD Diagnostic Criteria
PTSD Symptom Category Symptoms
Re-experiencing 1. Recurrent and intrusive thoughts
(at least 1 symptom) 2. Recurrent and distressing dreams of the trauma
3. Intense psychological distress at cues that recall or symbolize
the traumatic event
4. Physiological reactivity on exposure to cues that recall or sym-
bolize the traumatic event
5. Flashbacks
Avoidance 1. Avoiding thoughts, feelings or conversations associated with the
trauma
(at least 3 symptoms)
2. Avoiding activities, places or people associated with the trauma
3. Markedly diminished interest or participation in significant activities
4. Feelings of detachment or estrangement from others
5. Inability to recall important aspects of the trauma
6. Restricted range of affect
7. Sense of foreshortened future
Increased Arousal 1. Difficulty falling or staying asleep
(at least 2 symptoms) 2. Difficulty concentrating
3. Irritability or outbursts of anger
4. Hypervigilance
5. Exaggerated startle response
Specific Phobia
There is marked and persistent fear that is excessive and unreasonable cued by the
presence or anticipation of a specific object or situation (e.g., flying, heights, animals,
injections, seeing blood)
Exposure to the stimulus provokes an immediate anxiety response, which may be a
situationally bound panic attack (unlike panic disorder, where panic attacks are unexpected)
Realization that the fear is excessive or unreasonable
Phobias are among the most common of all psychiatric disorders, affecting 5 to 10% of the
population
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Treatment
Behavioral therapy
Create hierarchy of fears
Prevent patient from completing the compulsive behaviors
Medications
FDA approved SSRIs: Fluvoxamine, fluoxetine, sertraline, paroxetine
FDA approved TCA: Clomipramine
Neurosurgery for severe cases
Deep brain stimulation (DBS)
Bilateral cingulotomy
Dysthymic Disorder
Depressed mood for most of the day, for more days than not, for at least 2 years
Presence of 2 of the following:
Poor appetite or overeating
Insomnia or hypersomnia
Low energy or fatigue
Low self-esteem
Poor concentration or difficulty making decisions
Feelings of hopelessness
Often described as a chronic, low-grade depression
Epidemiology
Lifetime prevalence: 6%
More frequent in females2:1
Treatment
Similar to MDD
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8.3 BIPOLAR DISORDERS
Bipolar I Disorder
Occurrence of 1 or more manic or mixed episodes
Episodes of depression frequently occur but are not required for the diagnosis
Manic Episode
1 week (or any duration if hospitalized) of abnormally Mnemonic: DIG FAST
and persistently elevated, expansive, or irritable mood, D: Distractibility
together with 3 (4 if the mood is irritable) of the following I: Impulsive pleasurable activities
G: Grandiosity
symptoms:
F: Flight of ideas
Inflated self-esteem or grandiosity A: Activities towards goals
Decreased need for sleep S: Sleep
More talkative T: Talkativeness
Bipolar II Disorder
1 major depressive episode and at least 1 hypomanic episode
Hypomanic Episode
Criteria are the same than for manic episode except for:
Episode lasts at least 4 days
Episode is not severe enough to cause marked impairment in social or occupational
functioning, or to necessitate hospitalization, and there are no psychotic features
Treatment
Similar to bipolar I
Somatization Disorder
Multiple recurrent somatic complaints starting before age of 30
Pain symptoms in at least 4 sites, and 2 or more GI symptoms, 1 sexual or reproductive
symptom, and 1 pseudoneurological symptom
The disorder usually results in significant medical diagnostic testing, medical intervention
and other iatrogenic problems
Treatment
Regularly scheduled appointments with the primary care physician to lessen frequency of
ER visits and doctor shopping
Screen for and treat co-morbid psychiatric disorders
Conversion Disorder
Symptoms present as voluntary motor or sensory dysfunction suggestive of a neurologic or
other general medical condition
Psychological factors are prominent, and the initiation or exacerbation of the symptoms is
preceded by conflicts or stressors
The symptoms are not fully explained after clinical assessment as a medical condition or
culturally sanctioned phenomenon
Common symptoms include sudden paralysis, abnormal movements, aphonia, blindness,
deafness, or pseudoseizures
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Treatment
Reassure the patient and inquire about stressors
Focus on regaining function and suggest the course of impairment is brief
Direct confrontation is not recommended
Screen for domestic violence
Hypochondriasis
Preoccupation with fears of having a serious disease based on the patients
misinterpretation of bodily symptoms for at least 6 months
Preoccupation persists despite appropriate medical evaluation and reassurance
Preoccupation is not delusional, not consistent with body dysmorphic disorder, and is not
better accounted for by another psychiatric disorder
Treatment
Manage psychological stressors in collaboration with a psychiatrist
Reassure and validate distress
Pain Disorder
Pain is the prominent clinical presentation for at least 6 months
Psychological factors have a significant role in the outset, severity, exacerbation, or
perpetuation of the pain syndrome
The pain is not a component of somatization disorder
Co-morbid MDD is extremely common
Treatment
Refer to multidisciplinary pain clinic
Cognitive and biofeedback therapy
TCAs for pain and comorbid psychiatric conditions
Limit inappropriate use of analgesics
Anorexia Nervosa
Diagnostic Criteria (Must Meet All 4 Criteria)
1. Refusal to maintain normal weight for age and height (less than 85% of ideal weight)
2. Intense fear of gaining weight
3. Disturbance in the way body weight or shape is experienced or denial of the seriousness
of the current low body weight
4. Amenorrhea in postmenarcheal females who are not on OCPs
Types
Restricting type (limits food intake to avoid weight gain)
Binge-eating/purging type (self-induced vomiting or misuse of laxatives or diuretics)
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Table 8.5: Medical Complications of Anorexia Nervosa
Bradycardia Depletion of fat
Hypotension Muscle wasting (including cardiac muscle in severe
wasting)
Hypothermia
Lanugo (fine hair of the body)
Hypokalemia
Hypercortisolemia
Arrhythmias and sudden death
Osteoporosis
Leukopenia
Treatment
Hospitalization to reestablish weight and correct metabolic abnormalities
Behavioral modification, cognitive behavioral therapy, family therapy
SSRI for co-morbid depression
Bulimia Nervosa
Diagnostic Criteria
Recurrent episodes of binge eating characterized by both:
1. Eating, in a discrete period of time, an amount of food that is definitely larger than
most people would eat during a similar circumstances
2. A sense of lack of control over eating during the episode
Recurrent inappropriate compensatory behaviors to prevent weight gain: Self-induced
vomiting, diet pills, fasting, misuse of laxatives, diuretics, enemas, and or excessive
exercise
2 episodes of binge eating and purging/severe compensatory behaviors per week for a
minimum of 3 months
Types
Purging type (self-induced vomiting or the misuse of laxatives, diuretics, or enemas)
Nonpurging type (fasting or excessive exercise)
Treatment
Cognitive behavioral therapy
SSRIs treat binge-eating component, regardless of whether there is comorbid depression
Types/Diagnostic Criteria
Diagnosis is made clinically, supported by reports (scales) from teachers and parents
Some symptoms must have been present before the age of 7
There has to be evidence of impairment in social, academic or occupational functioning.
Impairment MUST be present in at least 2 settings (school/home/work)
Symptoms must have persisted for at least 6 months
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Treatment
First-line: Stimulants: Amphetamines (e.g., Adderall) and methylphenidate (e.g., Ritalin,
Focalin, Concerta)
Most common side effects of stimulants: Decreased appetite and insomnia. Usually
managed with clonidine or guanfacine
Also first-line: Atomoxetine (Strattera), a norepinephrine reuptake inhibitor
Atomoxetine usually started if anxiety is a prominent feature. It is less associated with
insomnia or decreased appetite
Second-line: -2 receptor agonists (clonidine and guanfacine) and bupropion
SSRIs usually started for comorbid depression or anxiety
Psychotherapy recommended when behavioral problems, depression, or anxiety are
prominent features
8.7 SCHIZOPHRENIA
2 of the following symptoms for 1 month:
Delusions (false fixed beliefs resistant to reason or confrontation and not explained by
patients culture)
Hallucinations (false sensory perceptions in the absence of external stimulus)
Disorganized speech
Grossly disorganized or catatonic behavior
Negative symptoms (affective flattening, alogia, or avolition)
Continuous signs of the disturbance persist for at least 6 months
Epidemiology
Prevalence: 1% worldwide
Men=women
Peak ages of onset: 18 to 25 for males, 25 to 35 years for females
Etiology
Increased dopaminergic activity
Serotonin and glutamate also involved
Psychiatric Comorbidities
Nicotine dependence: 80 to 90%
Anxiety disorders (OCD, panic disorder)
Schizotypal, schizoid, and paranoid personality disorder
Pharmacological Treatment
Typical or atypical antipsychotics (see Table on Common Antipsychotics)
Types
Autistic Disorder
Affects all 3 major areas seen in the PDD
Delays must be present before the age of 3
No specific laboratory test for autism but conditions such as Fragile X and tuberous
sclerosis should be excluded
Diagnosis is made clinically, supported by complete history of pregnancy, neonatal, and
developmental history, along with scales provided to parents
Chief complaint is usually a concern due to unusual sensitivities to the environment (e.g.,
light, sounds, labels of clothes) or that child might be deaf
Language is typically significantly delayed or absent in the 1st years
Unusual behaviors (e.g., stereotyped movements, extreme rigidity with schedules) are
common
Prevalence: 1 in 1,000
3 to 4 times more common in males than females, but in females it tends to be more
associated to severe mental retardation
50% have severe-profound mental retardation; 30% mild-moderate; 20% have normal IQ
Retts Disorder
Early growth and development is normal followed by a deceleration in head growth after
the age of 5 months. The only PDD with decreased life expectancy
Loss of previously acquired skills in language, social skills, and hand skills
Patients develop stereotyped hand movements (wringing, washing-like movements)
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Aspergers Disorder
Similar to autistic disorder but there is no significant delay in language or cognition
Social deficits become more prominent as the child enters preschool
PDD-Not Otherwise Specified (NOS)
Impairment in all 3 areas (social, language, and stereotyped behavior) but not enough to
meet criteria for one of the other PDDs
Childhood Disintegrative Disorder
Apparently normal development for the 1st 2 years of life, followed by a marked
regression in development (language, social, play, motor skills) before the age of 10
Treatment
There are no specific medications for PDDs and no medication treats the core symptoms.
Management depends on the clinical presentation:
If anxiety is present, patients might require use of SSRIs
If behavioral problems/aggressive behaviors are present, atypical antipsychotics
including risperidone can be used
Lithium, anxiolytics (e.g., benzodiazepines) and mood stabilizers have been used in
these patients with varying degrees of success
Structured environment and behavioral methods are most effective
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Table 8.10: Common Antipsychotics
Type Medication Name Daily Dose Side Effects *Comments
Range (mg)
1st-generation Haloperidol 1-20 Acute dystonia
antipsychotics Akathisia
(typicals)
Tardive dyskinesia
Hyperprolactinemia
Chlorpromazine 100-1,000 Sedation
Hypotension
Q-T interval prolongation
Decrease in seizure threshold
Anticholinergic effects
2nd-generation Aripiprazole 10-30 Headache
antipsychotics Somnolence
(atypicals)
Akathisia
Clozapine 50-900 Sedation
Orthostatic hypotension
Metabolic syndrome
Agranulocytosis (requiring frequent labs)
Hypersalivation
Dose-dependent seizures
*Used in treatment-resistant schizophrenia
*Decreases suicidal risk
Olanzapine 5-20 Metabolic syndrome
Quetiapine 50-800 Somnolence
Risperidone 1-6 Extrapyramidal symptoms
Hyperprolactinemia
Ziprasidone 40-160 Q-T interval prolongation
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Medication Induced Syndromes
Serotonin Syndrome
Due to:
Combination of SSRIs+ MAOI, L-tryptophan, or lithium
Overdose of MAOIs
Symptoms: Mental status changes, confusion, restlessness, hyperreflexia, tremor, ataxia,
nausea, diarrhea
Caution when using: Meperidine, tramadol, triptans, dextromethorphan
Treatment: Supportive measures, stop causing medications, benzodiazepines
Serotonin Discontinuation (or Withdrawal) Syndrome
Due to abrupt discontinuation of medication
Occurs within 48-hours of stopping antidepressant
Venlafaxine, fluvoxamine, and paroxetine have higher risk due to short half-lives
Symptoms include anxiety, irritability, nausea, headaches, insomnia, fatigue, dysphoria, flu-
like symptoms
Treatment: Fluoxetine at low dose (10-20 mg daily) for 1 to 2 weeks and then discontinue
Neuroleptic Malignant Syndrome (NMS)
A life-threatening reaction to an antipsychotic medication Mnemonic: FEVER
All antipsychotics may cause the syndrome, however, it is more F: Fever
frequently associated with potent antipsychotics (haloperidol) E: Encephalopathy
V: Vitals unstable
Some antiemetic medications such as metoclopramide can
E: Elevated CPK
cause the syndrome R: Rigidity
Treatment: Stop antipsychotic, cooling blankets, supportive
measures, muscle relaxants (dantrolene), or dopamine agonists
(bromocriptine, amantadine)
Re-challenging with the same medication will likely induce NMS again
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NOTES
Alexandra Voinescu, MD
C o n t e n t s
9.5 Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
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9.1 Clinical Evaluation of Kidney Function
Estimation of the Glomerular Filtration Rate (GFR)
The National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF K/DOQI)
chronic kidney disease (CKD) guidelines define CKD as either kidney damage (proteinuria,
hematuria, or structural abnormalities of the kidney) with or without impaired GFR, or
impaired eGFR <60 mL/min per 1.73m2 with or without kidney damage that persists for
over 3 months
Estimation of GFR is used clinically to assess the degree of kidney impairment and to
measure disease progression to determine prognosis and effects of treatment
The GFR is equal to the sum of the filtration rates in all of the functioning nephrons. Thus,
the GFR gives a rough measure of the number of functioning nephrons
Measurement of GFR is complex, time consuming, and cumbersome to do in routine clinical
practice. Although GFR cannot be measured directly, the best method for estimating GFR
is measurement of the urinary clearance of an ideal filtration marker
Inulin is the gold standard filtration marker, as it is freely filtered at the glomerulus, and is
neither secreted, reabsorbed, synthesized, nor metabolized by the kidney
Other less cumbersome methods for measuring clearance have been developed, using
alternative exogenous markers, such as iothalamate, iohexol, DTPA, or EDTA
Serum Creatinine
The most widely used indirect measure of GFR
Creatinine, a metabolic product derived from the metabolism of creatine in the skeletal
muscle and from dietary meat intake, is released into circulation at a relatively constant
rate
Creatinine is freely filtered in the glomeruli, and is not reabsorbed or metabolized by the
kidney
10 to 30% of the creatinine in the bloodstream is not filtered in the glomeruli, but is
secreted in the urine by the organic cation secretory pathways in the proximal tubules
Because production of creatinine is constant, the serum creatinine level depends on the
rate of clearance, which mainly reflects the GFR
There are many limitations of using creatinine to estimate GFR. A reduction in muscle
mass (e.g., malnutrition, liver failure, or muscle wasting) can cause a disproportionately
low creatinine level that results in overestimation of the GFR. Early in the course of
chronic kidney disease, there is an enhanced tubular creatinine secretion by as much as
50%, which may result in overestimation of the GFR. In addition, creatinine secretion can
be blocked by different drugs (e.g., trimethoprim, cimetidine), resulting in a rise in the
serum creatinine of as much as 0.4 to 0.5 mg/dL and an underestimation of the GFR
Creatinine Clearance
Usually determined from a 24-hour urine collection
Limitations of using creatinine clearance include inaccurate urine collection and variation in
the degree of creatinine secretion, which increases as GFR decreases
The accuracy of 24-hour urine collection can be estimated from comparing the total urine
creatinine excretion with the normal rate of creatinine excretion (20 to 25 mg/kg/24 h in
men, and 15 to 20 mg/kg/24 h in women)
Interpretation of Urinalysis
Dipstick Analysis
Specific Gravity
Reflects the relative proportion of dissolved solutes to total volume
Depends on both the number and weight of the dissolved particles
Normal range is 1.003 to 1.030
Values decrease with age as the kidneys ability to concentrate urine decreases
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Is also influenced by protein, glucose, mannitol, diuretics, radiocontrast media, and some
antibiotics
Low values (<1.010) are consistent with dilute urine, which may be seen in overhydration
or diabetes insipidus
High values (>1.030) are consistent with concentrated urine
pH
The physiologic urine pH ranges between 4.5 to 8.0
A very alkaline urine (pH >7.0) is associated with urinary tract infections with a urea-
splitting organism, vegetarian diet, metabolic alkalosis
A low urine pH (pH <5.0) is associated with metabolic acidosis and with the ingestion of
large amounts of meat
A urine pH above 5.5 in the setting of metabolic acidosis suggests impairment in the
acidification process, indicating the presence of 1 of the forms of renal tubular acidosis
Glucose
Glycosuria occurs in the presence of uncontrolled hyperglycemia, when the filtered
glucose load is increased to a level that exceeds proximal glucose reabsorptive capacity
The renal threshold for glucose is generally 160 to 180 mg/dL
Renal glycosuria, associated with normal plasma glucose concentration, is seen with a
defect in proximal tubular reabsorption of glucose, such as Fanconi syndrome
Ketones
The dipstick reveals the presence of acetoacetate and acetone, but not the beta-
hydroxybutyrate (often 80% of total serum ketones in ketosis)
Urine ketones are associated with diabetic and alcoholic ketoacidosis, prolonged fasting
or starvation
False positive results may occur in patients taking different drugs, such as levodopa or
drugs that contain free sulfhydryl groups (e.g., captopril, mesna)
Leukocyte Esterase
Detects the presence of leukocytes in the urine
The test threshold is 5 to 15 white blood cells per high-power field (WBCs/hpf)
False positive results can occur with vaginal contamination
False negative results can occur with glycosuria, vitamin C, cephalexin and tetracycline
therapy, or excessive oxalate excretion
Nitrite
Dietary nitrates are normally excreted in the urine
The dipstick reveals the presence of bacteria which have the capability of reducing
nitrates to nitrites due to nitrate reductase activity, which is present in most gram-
negative urinary pathogens
False negative results occur in the presence of organisms that do not produce nitrite,
when vitamin C is present, or when urine has not been retained in the bladder long
enough (approximately 4 hours) to permit sufficient production of nitrite from nitrate
Bilirubin
Only conjugated bilirubin is passed into the urine
The dipstick is positive in patients with jaundice due to hepatobiliary disease, but is
negative in patients with jaundice due to hemolysis
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Leukocytes
Pyuria (presence of WBCs) is defined as increased amount of leukocytes in the urine
(>4/hpf)
Pyuria can arise from several conditions, by far the most common being infection
Sterile (culture-negative) pyuria can be seen in active tuberculosis infection, severe sepsis,
interstitial nephritis, and nephrolithiasis
Wright or Hansel staining of the urine sediment can reveal eosinophiluria, which may
occur in acute interstitial nephritis, in various types of glomerulonephritis, chronic
pyelonephritis, septic emboli, acute atheroembolic disease of the kidney
Epithelial Cells
Renal tubular cells derive from the exfoliation of the tubular epithelium
The presence of renal tubular cells, especially in association with casts, indicates a renal
source of injury, such as acute tubular necrosis, pyelonephritis, acute interstitial nephritis,
and acute cellular rejection
Casts
Are cylindrical bodies that form in the distal tubules and collecting tubules where Tamm-
Horsfall glycoprotein precipitates and entraps cells present in the urinary space
Hyaline casts and fine granular casts can be seen in normal individuals and in patients
with renal disease
Coarse granular casts, result from degeneration of embedded cells, are nonspecific but
usually pathologic
Waxy casts are believed to result from the degeneration of cellular casts, and, thus, can
be seen in a variety of kidney disease
Red blood cell casts are a hallmark of proliferative glomerulonephritis
White blood cell casts are seen in pyelonephritis, acute interstitial nephritis, but can also
be seen in glomerulonephritis
Pigmented casts derive their distinctive color from bilirubin or hemoglobin and are found
in hyperbilirubinemia or hemoglobinuria
Crystals
Many different forms of crystals can be observed in normal patients and in those with
defined diseases
Uric Acid Crystals
Are found only in acid urine, a milieu that favors the conversion of the relatively soluble
urate salt into the insoluble uric acid
May have different forms, including rhomboidal, rectangular, or needles
Calcium Oxalate Crystals
The formation of these crystals is not dependent upon the urine pH
Are small, uniform, described as envelope shape
Calcium Phosphate
Only form in a relatively alkaline urine
Are usually narrow rectangular needles
Cystine Crystals
Precipitate only in acid urine
Have a hexagonal shape
Are diagnostic of cystinuria
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Radionuclide Scans
Can be used to calculate glomerular filtration rate (GFR) and effective renal plasma flow
(ERPF), even in cases of impaired renal function
Captopril renogram is a screening test for renovascular disease
The diuretic renogram can be obtained in cases of suspected obstructive uropathy
Voiding Cystourethrography
Can be used to detect vesicoureteral reflux
Retrograde or Anterograde Pyelography
May be used to diagnose urinary tract infections
Kidney Biopsy
Kidney biopsy is performed when histologic confirmation is needed to help diagnose and
treat different kidney diseases
Kidney biopsy is indicated in patients with nephrotic syndrome, in patients with renal
disease in the setting of a systemic disorder, in any patient with acute renal failure in
whom the diagnosis in not clear, and in renal transplant dysfunction. Some patients with
non-nephrotic proteinuria associated with hematuria, and patients with unexplained
chronic kidney disease may also benefit
Renal biopsy is often performed into the lower pole of kidney under direct ultrasound or
CT guidance
Complications of renal biopsy include pain (particularly if a large perinephric hematoma
develops), gross hematuria (1.5% of cases) and the need for blood transfusion (1%).
If hemorrhage is severe and prolonged, angiography should be used to identify the
feeding artery, which can be embolized. Other complications include arteriovenous
fistula, and Page kidney, complication in which subcapsular hematoma compresses the
kidney provoking ischemia and high renin hypertension; surgical decompression may be
necessary
Contraindications to renal biopsy include uncontrolled blood pressure, uncontrolled
bleeding diathesis, uncooperative patient, multiple renal cysts, acute pyelonephritis, renal
neoplasm, and solitary kidney
Most contraindications to renal biopsy are relative rather than absolute
Evaluation of Hyponatremia
Plasma osmolality
Low: True hyponatremia
Normal or high
Urine osmolality
Less than 100 msom/kg: Primary polydipsia, reset osmostat
More than 100 mosm/kg: Other causes of true hyponatremia
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Urine sodium concentration
Less than 25 meq/L: ECV depletion
More than 40 meq/L: SIADH, diuretics, renal failure
Symptoms of Hyponatremia
Clinical manifestations of acute hyponatremia include neurological symptoms induced by
cerebral edema
Nausea and malaise may be seen at a serum sodium concentration of 125 to 130 meq/L
Headache, lethargy, seizures, and coma may occur at a serum sodium concentration of 115
to 120 meq/L
Due to cerebral adaptation, neurological symptoms are less severe in the setting of chronic
hyponatremia, and include the following: Fatigue, nausea, gait disturbances, and attention
deficit
Treatment of Hyponatremia
Symptomatic Hyponatremia
Acute, life-threatening hyponatremia requires an aggressive treatment with hypertonic
saline
Usually 4 to 5 meq/L increase in serum sodium concentration is adequate to prevent
permanent neurologic sequelae
Dose: 100 mL or 2 mL/kg bolus infusion of 3% saline, repeated up to two times if
necessary
Loop diuretics can also be added in this setting
Patients with mild and moderate neurological symptoms may require hypertonic saline,
but there is no need for an aggressive approach
The goal is an increase in serum sodium concentration of less than 10 meq/L in the 1st
24 hours and less than 18 meq/L in the 1st 48 hours
Patients with symptomatic hypovolemic hyponatremia can be treated with 0.9% NaCl
An overly rapid increase in the serum sodium concentration can lead to osmotic
demyelination
Asymptomatic Hyponatremia
Hypovolemic hyponatremia: Isotonic 0.9% NaCl
Hypervolemic hyponatremia: Fluid restriction with and without loop diuretic therapy, IV
vaptans
Normovolemic hyponatremia: Fluid restriction, demeclocycline, tetracycline antibiotic,
which antagonizes the action of ADH, vaptans
Hypernatremia
Hypernatremia is defined as a serum sodium concentration greater than 145 meq/L, and
represents a deficiency of total body water relative to total body sodium
Etiology of Hypernatremia
Water Loss
Insensible losses
Increased sweating: Fever, exercise
Burns
Respiratory losses
Renal losses
Central diabetes insipidus
Nephrogenic diabetes insipidus
Osmotic diuresis
Gastrointestinal losses
Osmotic diarrhea
Hypothalamic disorders
Primary hypodipsia
Water loss into cells
Severe exercise, seizures, rhabdomyolysis
Sodium Retention
Administration of hypertonic NaCl or NaHCO3
Ingestion of NaCl tablets
Assessment of Polyuria
Polyuria is generally defined as a urine volume above 2.5 L/d
Polyuria is mediated by excessive excretion of water (water diuresis) or solutes, such as
sodium, urea, glucose (solute diuresis)
Water diuresis (urine osm <150 mosm/kg)
Primary polydipsia
Central diabetes insipidus
Nephrogenic diabetes insipidus
Solute diuresis (urine osm >300 mosm/kg)
Saline loading
Post-obstructive diuresis
Hyperglycemia
High-protein tube feedings
Renal salt wasting
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Diabetes insipidus
Central diabetes insipidus (CDI) is caused by inadequate secretion of AVP from the
posterior pituitary gland in response to osmotic stimulation
Nephrogenic diabetes insipidus (NDI) is associated with resistance to the antidiuretic
action of AVP due to a defect within the kidney
Causes of CDI
Neurosurgical procedures
Head trauma
Hypoxic encephalopathy
Malignancy: Primary (craniopharyngioma) or metastatic (breast, lung)
Meningitis, encephalitis
Pituitary infiltrative diseases (histiocytosis X, sarcoidosis)
Idiopathic
Causes on NDI
Electrolytes abnormalities (hypokalemia, hypercalcemia)
Drugs: Demeclocycline, lithium, ifosfamide, foscarnet, amphotericin B
Chronic tubulointerstitial disease
Pregnancy
CDI and NDI are both characterized by polyuria and polydipsia as well as an absence
of change in urine osmolality in response to water deprivation. Subcutaneous
administration of antidiuretic hormone causes an increase in urine osmolality in
patients with CDI but causes no change in urine osmolality in patients with NDI
Treatment of Hypernatremia
Treat underlying disease
Patients with evidence of hemodynamic collapse should receive isotonic fluids (normal
saline) until hemodynamically stable
Calculate free water deficit
Free water deficit = TBW x (plasma Na/140 1)
Correction should occur slowly; aim for a decrease in serum sodium of 0.5 meq/L/hour
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Hyperkalemia
Defined as a potassium level of 5.5 mmol/L or higher
Causes of hyperkalemia
1. Pseudohyperkalemia can be caused by the mechanical release of potassium from cells
during phlebotomy or with severe thrombocytosis or leukocytosis due to release of
potassium from these cellular elements
2. Excessive dietary intake
3. Cellular redistribution
Tissue damage (rhabdomyolysis, trauma, burns, tumor lysis syndrome, hemolysis)
Metabolic acidosis (mineral acidosis associated with ammonium chloride or
hydrochloric acid results in potassium efflux from cells because of the relative
impermeability of the chloride anion)
Severe exercise
Drugs: Succinylcholine, digitalis, beta-adrenergic blockade
Insulin deficiency and hyperglycemia (potassium movement via solvent drag)
4. Decreased urinary excretion
Renal failure
Effective circulating volume depletion
Hypoaldosteronism state
Drugs: NSAIDs, ACEI, ARB, renin inhibitors, cyclosporine and tacrolimus, amiloride,
triamterene, spironolactone, heparin
Clinical Manifestations
Muscle weakness, paralysis and respiratory failure
Cardiac arrhythmias
Electrocardiographic findings in patients with hyperkalemia include peak T waves,
lengthening of the PR and QRS intervals, development of a sine wave, ventricular
fibrillation and asystole
Treatment of Hyperkalemia
Stabilization of excitable membranes with intravenous calcium gluconate
Increased potassium entry into the cells with insulin, beta adrenergic agonists, or sodium
bicarbonate
Removal of the potassium: Potassium wasting diuretics, cation-exchange resin
(Kayexalate), dialysis
Treatment
Phosphate repletion is indicated when serum phosphate level is less than 2 mg/dL
Intravenous phosphorus repletion is reserved for patients with severe hypophosphatemia
(<1 mg/dL)
Intravenous phosphate is potentially dangerous, since it can precipitate with calcium and
produce various adverse effects, including hypocalcemia, renal failure due to calcium
phosphate precipitation, and potentially fatal arrhythmias
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The bicarbonate concentration is calculated using the Henderson equation:
H+ = 24 x PCO2/HCO3-
The total serum CO2 is directly measured from venous blood specimen and should
be compared to the calculated bicarbonate to ensure accuracy
2. Is the pH low or high?
pH <7.4 indicates acidosis
pH >7.4 indicates alkalosis
3. Is the primary disturbance metabolic (HCO3) or respiratory (PCO2)?
Look at the PCO2 and serum CO2 to see which one is consistent with the pH, and
to determine if the primary disorder is respiratory or metabolic
4. Is compensation appropriate?
Yes simple acid-base disorder
No mixed acid-base disorder
Metabolic Acidosis
Characterized by a fall in serum bicarbonate concentration, and is normally compensated
by increased ventilation, with decrease in PCO2
Can be caused by loss of HCO3- stores (diarrhea, renal tubular acidosis), increase in
endogenous production of acid (lactic acid, ketoacids), or progressive accumulation of
endogenous acids (renal failure)
The anion gap (AG) is a useful tool in the differential diagnosis of metabolic acidosis
+ - -
AG = Na - (Cl + HCO3 )
AG represents the unmeasured anions that are normally present in serum and
unaccounted for by the serum electrolytes measured on the electrolyte panel
The normal AG is 12 +/- 2
The unmeasured anions present in serum include phosphates, sulfates, organic acids,
and negatively charged plasma proteins
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Hyperkalemic Distal RTA
Results from aldosterone deficiency or resistance
There is both impaired hydrogen secretion and decreased urine ammonium excretion
May be caused by diabetes mellitus, interstitial nephritis, and drugs (spironolactone,
amiloride, triamterene, or cyclosporine)
Metabolic Alkalosis
A primary acid-base disturbance characterized by increased plasma bicarbonate level with
an increase in PCO2 as a result of compensatory alveolar hypoventilation
The maintenance of metabolic alkalosis represents a failure of the kidney to eliminate
bicarbonate at the normal capacity
Causes of metabolic alkalosis:
Exogenous administration of bicarbonate (administration of sodium bicarbonate, milk-
alkali syndrome, massive blood transfusion)
Acid loss
Gastrointestinal loss: Vomiting, nasogastric suctioning
Renal loss: Diuretics, administration of nonresorbable anions (e.g., intravenous
penicillin or carbenicillin), Bartter and Gitelman syndrome
Mineralocorticoid state
Measurement of a spot urine chloride is a more reliable index of the volume status than the
urinary sodium concentration
Urine chloride <25 meq/L: Vomiting, nasogastric suction, diuretics (late)
Respiratory Acidosis
The primary defect in respiratory acidosis is an increase in PCO2
Causes of respiratory acidosis:
Central: Drugs (sedatives, narcotics), respiratory center hypoperfusion (infection,
ischemia), obstructive sleep apnea
Lungs/airways: Obstruction/asthma, pneumonia, pulmonary edema, COPD, ARDS,
barotraumas
Neuromuscular: Poliomyelitis, kyphoscoliosis, muscular dystrophy, multiple sclerosis
Prediction of HCO3 in respiratory acidosis
HCO3 increases by 1 meq/L for each 10 mmHg increase in PCO2 (acute)
HCO3 increases by 4 meq/L for each 10 mmHg increase in PCO2 (chronic)
Respiratory Alkalosis
Characterized by a decrease in PCO2
Causes of respiratory alkalosis:
CNS stimulation: Anxiety, pain, fever, cerebrovascular accident, tumor, meningitis,
encephalitis
Hypoxemia: High altitude, pneumonia, pulmonary edema, aspiration, pulmonary
embolus
Drugs or hormones: Salicylates, progesterone, nicotine, xanthenes
Stimulation of chest receptors: Pneumonia, pneumothorax, pulmonary embolism
Others: Pregnancy, liver failure, septicemia, mechanical hyperventilation
Prediction of HCO3 in respiratory alkalosis
HCO3 decreases by 2 meq/L for each 10 mmHg decrease in PCO2 (acute)
HCO3 decreases by 4 meq/L for each 10 mmHg decrease in PCO2 (chronic)
Treatment of respiratory alkalosis is directed at correcting the primary cause. In addition,
sedation or rebreathing into a paper bag can increase the arterial PCO2, resulting in partial
correction of hypocapnia
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There are many causes of primary as well as secondary causes of chronic tubulointerstitial
nephritis
7. Obstructive disorders
3. Hematologic disorders
Tumors
Sickle cell disease
Nephrolithiasis
Light chain disease
Vesicoureteral reflux
Amyloidosis
Lymphoproliferative disease
4. Metabolic disorders
Hypercalcemia/nephrocalcinosis
Hypokalemia
Hyperuricemia
Hyperoxaluria
Cystinosis
Secondary
Secondary chronic tubulointerstitial nephritis includes diseases in which the primary pathogenic process
begins in the glomerular, vascular, or tubular systems followed by damage to the tubulointerstitial
compartment
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Analgesics
Analgesic nephropathy is responsible for 20% of cases of tubulointerstitial disease
Analgesic nephropathy develops in patients who abuse the compound analgesics
containing aspirin or antipyrine, combined with phenacetin, acetaminophen (paracetamol)
or salicylamide, and caffeine and codeine
Many studies suggest a dose-dependent risk for developing analgesic nephropathy with
compound analgesics; the total amount of phenacetin necessary to cause analgesic
nephropathy >3.0 kg
The main site of renal injury is medulla, which becomes vulnerable because of the
concentration of toxic metabolites built up by the counter-current mechanism and
because of the low oxygen tension present in this region
Aspirin and NSAIDs can result in the reduction of vasodilatory prostaglandins, with
decreased local renal blood flow within the medulla
Phenacetin is converted to acetaminophen, which causes cellular depletion of
glutathione, and results in the formation of oxidative and alkylating metabolites
Caffeine can be metabolized to adenosine, with vasoconstrictive effects within the
kidney
The result is papillary ischemia and eventually papillary necrosis
Patients with analgesic nephropathy present with slowly progressive chronic kidney
disease, with bland urinalysis or with sterile pyuria and/or mild proteinuria (usually less
than 1.5 g/d)
Intravenous pyelography shows papillary necrosis when part or all of the papilla has
sloughed
CT scan without contrast shows bilateral reduced renal size, bumpy renal contours, and
bilateral papillary calcifications (small, indented calcified kidneys)
Analgesic nephropathy is associated with increased risk of transitional cell carcinoma of
the uroepithelium. This should be further investigated especially in patients with gross
hematuria
Other causes of papillary necrosis can be remembered by the mnemonic POSTCARD
(Pyelonephritis, Obstruction, Sickle cell disease or trait, Tuberculosis, Chronic alcoholism
with cirrhosis, Analgesics, Renal vein thrombosis, and Diabetes mellitus)
Lithium
Chronic lithium ingestion has been associated with polyuria, polydipsia, and nephrogenic
diabetes insipidus, and less frequently, with chronic tubulointerstitial nephropathy
Nephrogenic diabetes insipidus appears to result from lithium accumulation in collecting
tubule cells after entering these cells through sodium channels in the luminal membrane,
interfering with the ability of antidiuretic hormone (ADH) to increase water reabsorption
by inhibiting adenylate cyclase and hence cAMP production, and also by decreasing the
apical membrane expression by aquaporin 2 water channels
In patients who develop nephrogenic diabetes insipidus, lithium therapy should be
discontinued, if possible. If lithium therapy is continued, concomitant amiloride therapy is
recommended, as amiloride can block lithium uptake through the sodium channels in the
collecting duct, and can reduce the urine output by up to 50%
15 to 20% of the patients on chronic lithium therapy develop chronic tubulointerstitial
disease
9.5 Hypertension
Epidemiology
Affects almost 30% of the adult population in the US
Is a common risk factor for cardiovascular disease and progressive chronic kidney disease
Less than 50% of hypertensive patients have adequate blood pressure control
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Definitions
The Joint National Committee (JNC 7) guidelines proposed the following definitions based
upon the average of 2 or more blood pressure readings obtained at 2 or more visits:
Normal blood pressure: Systolic <120 mmHg and diastolic <80 mmHg
Prehypertension: Systolic 120-139 mmHg or diastolic 80-89 mmHg
Hypertension as 140/90 mmHg or higher
Stage 1 hypertension: Systolic 140-159 mmHg or diastolic 90-99 mmHg
Stage 2: Systolic 160 or diastolic 100 mmHg
The definition of hypertension based upon ambulatory blood pressure monitoring:
A 24-hour average above 135/85 mmHg
Daytime (awake) average above 140/90 mmHg
Nighttime (asleep) average above 125/75 mmHg
Isolated systolic hypertension
Hypertension is only systolic, with normal diastolic blood pressure
It is usually seen in elderly patients and is a result of decreased arterial elasticity
Malignant hypertension is defined as marked hypertension with retinal hemorrhages,
exudates, or papilledema, and by evidence of renal damage
Risk Factors
Race (hypertension is more prevalent in African decent population)
Family history of hypertension in maternal, paternal, or both parents
Excess alcohol intake
High sodium intake
Obesity, weight gain, and physical inactivity
Dyslipidemia
Vitamin D deficiency
Complications
Left ventricular hypertrophy is seen commonly in patients with hypertension, and is
associated with increased risk of cardiovascular events, especially heart failure and stroke
Hypertension is the most common risk factor for ischemic stroke
Heart failure (patients with hypertension have a 3-fold higher risk of heart failure than
normotensive subjects)
Hypertension is a risk factor for chronic kidney disease and end-stage renal disease
Evaluation
Office Blood Pressure Measurement
Adequate measurement and interpretation of the blood pressure is essential in the
diagnosis and management of hypertension
In adults, classification of hypertension is based on an average of 2 or more readings
obtained 5 minutes apart, at 2 or more visits
Single office blood pressure measurement is not able to detect white coat hypertension,
masked hypertension, and labile hypertension
White coat hypertension is responsible to 20 to 25%of patients diagnosed with stage 1
office hypertension. Repeat blood pressure measurements are normal at home, at work,
or by ambulatory blood pressure monitoring
Management
The goal of treatment of hypertension is to reduce cardiovascular morbidity and mortality
by lowering blood pressure
Treatment of hypertension should involve both nonpharmacologic therapy (also called
lifestyle modification) and antihypertensive drug therapy for all patients with stage 1 or
greater hypertension
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Lifestyle modifications include the followings: Dietary salt restriction, the DASH diet
(increased intake of fruits and vegetables and low-fat dairy products), weight loss, physical
activity, limit daily alcohol intake (limit to less than 2 drinks per day for men and <1 drink for
women), and smoking cessation
Lifestyle modifications lower not only blood pressure, but can also modify additional
cardiovascular risk factors, such as obesity, diabetes, and dyslipidemia
Antihypertensive Drugs
Antihypertensive drugs should be started if the systolic pressure is persistently
140 mmHg and/or the diastolic pressure is persistently 90 mmHg despite attempted
nonpharmacologic strategy
Therapy with 2 antihypertensive drugs should be considered in patients with a baseline
blood pressure above 160/100 mmHg
Most antihypertensive agents reduce blood pressure by 15 to 20%, and all classes of
antihypertensive drugs decrease cardiovascular morbidity and mortality compared with
placebo
Some patients may have specific indications for use of certain antihypertensive drugs that
are unrelated to essential hypertension
If there is no specific indication, monotherapy is generally initiated with the following class
of medications: Thiazide diuretics, long-acting calcium channel blockers (most often a
dihydropyridine), and angiotensin-converting enzyme (ACE) inhibitors or angiotensin II
receptor blockers (ARBs)
ACEI and ARBs are indicated in patients with heart failure, systolic dysfunction, post-
myocardial infarction and in proteinuric kidney disease
Diuretics are used in patients with salt-sensitive hypertension and in patients with systolic
hypertension. In addition, aldosterone antagonists (spironolactoneor eplerenone) are
indicated in selected patients with advanced heart failure who have relatively preserved
kidney function
-blockers are used in patients with coronary artery disease, for rate control in patients
with atrial fibrillation, angina pectoris, or in patients with migraine headaches
Calcium channel blockers are used in patients with angina and in systolic hypertension.
The non-dihydropyridine calcium channel blockers (verapamil, diltiazem) can be given for
rate control in patients with atrial fibrillation
Calcium channel blockers and diuretics are more effective in patients who are likely
to be sodium sensitive, such as older patients, African decent patients, and those with
lower pretreatment plasma renin activity. On the other hand, ACE inhibitors or -blockers
may be more effective in younger patients, Caucasian patients, and those with higher
pretreatment plasma renin activity
a-blockers are given in patients with benign prostatic hyperplasia
Adverse effects (see Table on Antihypertensive Drugs - Adverse Effects)
Secondary Hypertension
Should be considered in patients who present with atypical clinical features and are
resistant to antihypertensive therapy
The most common causes of secondary hypertension include primary aldosteronism,
renovascular disease, and pheochromocytoma
Primary Aldosteronism
Primary aldosteronism consists of a heterogeneous group of disorders characterized
by hypokalemic alkalosis with potassium wasting, increased plasma aldosterone and
suppressed plasma renin activity
Primary aldosteronism affects approximately 10 to 15% of patients with hypertension
Screening is recommended in the following settings: Spontaneous hypokalemia,
hypertension with an adrenal mass found incidentally on a routine abdominal CT scan,
hypertension in first-degree relatives of patients with primary hyperaldosteronism
Diagnosis
Plasma aldosterone concentration (>15 ng/dL)
The aldosterone to renin ratio (ARR) (>20)
After correction of hypokalemia and adherence to a high-sodium diet for 3 days,
24-hour urine sodium >200 mEq and urine aldosterone >12 g/24 h
High-resolution CT scan or MRI should be performed to identify an adenoma
Adrenal vein sampling is the gold standard to establish a lateralizing adenoma or
hyperplastic gland. However, adrenal vein sampling is very difficult and highly operator
dependent
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Treatment
Laparoscopic adrenalectomy
Patients who are poor candidates for surgery should be treated medically with
spironolactone or eplerenone
Renovascular Disease
Renovascular hypertension is defined as hypertension secondary to narrowing of one or
more of the renal arteries
85% of patients have occlusive atherosclerotic renovascular disease
Screening for renovascular disease
Duplex ultrasound
Magnetic resonance angiography
CT angiography
Treatment: Surgical revascularization, percutaneous transluminal angioplasty with stent
placement, and medical therapy
Pheochromocytomas
Pheochromocytomas are catecholamine-secreting tumors that arise within the adrenal
glands, but approximately 10% are extra-adrenal (paraganglioma)
Overproduction of catecholamines can cause labile or paroxysmal hypertension,
diaphoresis, headache, palpitations, and anxiety
10% of patients with pheochromocytomas have metastatic disease, particularly to bone,
lungs, liver, and regional lymph nodes
Complications of pheochromocytomas include myocardial infarction, stroke, and
cardiovascular collapse
Diagnosis of pheochromocytoma includes the measurement of urinary catecholamines,
urinary and plasma metanephrines, clonidine suppression test, MRI or CT scanning which
can localize the tumor, and scintigraphy with 123-I-metaiodobenzylguanidine (MIBG), which
can be used to confirm positive findings on CT and MRI scanning
Treatment of choice is surgical resection
Preoperative and postoperative hypertension should be treated with a combination of alpha
and b-blockers
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Screening modalities for cerebral aneurysm include high-resolution CT
angiography (CTA) or magnetic resonance angiography (MRA)
Valvular heart disease include mitral valve prolapse and aortic regurgitation
Screening and Diagnosis
Among at risk individuals, renal ultrasonography is usually the initial modality used for
screening and diagnosis
Ultrasonographic criteria for the diagnosis of ADPKD for at-risk individuals from families
of unknowngenotype
Among individuals between 15 and 39 years of age, at least 3 unilateral or bilateral
cysts
Among individuals 40 to 59 years of age, 2 cysts in each kidney
Among individuals 60 years or older, 4 cysts in each kidney
Ultrasonographic criteria for the diagnosis of ADPKD for at-risk individuals for type 1
ADPKD
Among individuals between 15 and 30 years of age, at least 2 unilateral or bilateral
cysts
Among individuals 30 to 50 years of age, 2 cysts in each kidney
Among individuals 60 years or older, 4 cysts in each kidney
In the absence of a family history, more strict criteria is required for diagnosis. The
diagnosis should be strongly suspected in the presence of multiple and bilateral cysts
(arbitrarily defined as 10 or more cysts in each kidney) in the absence of findings
suggestive of a different renal cystic disease
Genetic testing
Can be done when the imaging results are equivocal and/or a definitive diagnosis is
required
Direct DNA analysis of the PKD1 and PKD2 genes can be performed by linkage or
sequence analysis
Treatment
Treatment with angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor
blockers (ARBs) may lower the blood pressure and slow the rate of progression of
ADPKD, especially in patients with proteinuria
No specific therapy has been proven to prevent or delay progression of ADPKD.
Promising specific therapies that are being evaluated include vasopressin V2 receptor
antagonists (which reduces renal epithelial cell intracellular cAMP levels), mTOR inhibitors,
somatostatin, and EGF receptor antagonists
Renal cyst infection requires prolonged courses of treatment (up to 4 weeks)
with antibiotics that penetrate the cysts, such as trimethoprim-sulfamethoxazole,
fluoroquinolones, chloramphenicol, or vancomycin
Pain related to cyst hemorrhage is usually treated with analgesics, bed rest, and hydration
Autosomal-recessive Polycystic Kidney Disease
An inherited disease characterized by cystic dilations of the renal collecting ducts and
congenital hepatic fibrosis
The estimated incidence of ARPKD is 1:10,000 to 1:40,000 births
ARPKD is caused by mutations in the PKHD1 gene on the chromosome 6 that encodes
for fibrocystin (polyductin)
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Noncystic Disorders
Alport Syndrome
Alport syndrome is a generalized, inherited disease of basement membrane that results
from mutations in genes encoding the alpha-3, alpha-4, and alpha-5 chains of type IV
collagen
These alpha IV collagen chains are normally located in various basement membranes of
the kidney, cochlea, and eye
X-linked Alport syndrome is the predominant form of the disease, accounting for
approximately 85% of patients and arises from mutation in the COL4A5 gene on the X
chromosome
Autosomal recessive disease occurs in 15% of cases and arises from mutations affecting
both alleles of COL4A3 and COL4A4
Autosomal dominant disease occurs in 5% of patients and arises from heterozygous
mutations in the COL4A3 or COL4A4 genes
Clinical Features
Renal manifestations
The cardinal finding is persistent microscopic hematuria
Some patients develop recurrent episodes of gross hematuria, often precipitated by
upper respiratory infections
The serum creatinine and blood pressure are normal, and proteinuria is absent in
early childhood
Progressive renal failure, hypertension, and significant proteinuria develop with time.
End stage renal disease develops in the second or 3rd decade of life
Patients with autosomal recessive disease have a similar clinical presentation and
course as those with X-linked disease, whereas, patients with autosomal dominant
disease generally have a slower decline in renal function
Bilateral sensorineural hearing loss is common in Alport syndrome
Ocular defects
Anterior lenticonus is a forward protrusion of the anterior surface of the ocular lens
Corneal changes include posterior polymorphous dystrophy and recurrent corneal
erosions
Retinal flecks are small yellow or white dots scattered around the macula or in the
periphery of the retina
Diagnosis
Can be made by either a skin biopsy or a renal biopsy
Routine immunofluorescence examination for immunoglobulins and complement
components is negative, but staining for the alpha 5 (IV)-chain can be done and shows
no staining of the GBM with the alpha 5 antibody in X-linked males
Electron microscopy shows variable thickening, thinning, basket-weaving, and lamellation
of the GBM
Thin Basement Membrane Nephropathy (Benign Familial Hematuria)
An inherited disorder caused by several mutations of the type IV collagen genes COL4A3
and COL4A4
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The Acute Kidney Injury Network (AKIN) proposed the term acute kidney injury (AKI) to
represent the entire spectrum of acute renal failure. The proposed diagnostic criteria are an
abrupt increase in the serum creatinine concentration of at least 0.3 mg/dL from baseline,
or a percentage increase in serum creatinine concentration of 50% over a period of less
than 48-hours, or oliguria of less than 0.5 mL/kg per hour for more than 6 hours
Epidemiology
Incidence of acute kidney injury: 500/100,000 person years
Occurs in 5 to 10% of hospitalized patients and up to 20% of ICU admissions
Patients with acute kidney injury have an increased long-term risk for CKD progression or
death compared with hospitalized patients without AKI
The FENa and the urine sodium concentration are difficult to interpret with concurrent
diuretic therapy. The fractional excretion of urea (FEurea) may be most useful in this setting
Imaging studies: Renal ultrasound is indicated in all patients with AKI to define kidney
anatomy and echogenicity and to rule out hydronephrosis
Renal biopsy should be considered when the diagnosis remains unclear after ruling out
prerenal and postrenal causes. Biopsy if glomerulonephritis is suspected
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High urine sodium concentration (usually >40 meq/L) is caused by tubular injury and
decreased sodium reabsorption
High urine fractional excretion of sodium (FENa >2%)
FEUrea is between 50 to 65%
Urine osmolality <450 mOsm/kg
Urine creatinine/plasma creatinine ratio <20 (measure of tubular water reabsorption)
Treatment
Supportive care, hemodynamic support
Loop diuretics have not shown any benefit in preventing AKI or improving outcomes
in oliguric patients, but are routinely used in management of fluid overload and
hyperkalemia before initiation of renal replacement therapy
Several different agents have been studied in an attempt to prevent or ameliorate AKI,
including renal vasodilators, adenosine antagonists, or free radical scavengers. However,
no strategy showed improved benefit in improving outcomes or mortality
Contrast-induced Nephropathy
Defined as a sudden decline in kidney function within 24 to 48-hours of IV contrast
exposure
Kidney injury is usually non-oliguric
Creatinine peaks within 7 days and usually returns to baseline within 10 days
Caused by renal vasoconstriction leading to medullary ischemia, direct tubular
cytotoxicity of contrast, and the generation of reactive oxygen species, which contribute
to cell damage
Risk Factors
Underlying renal insufficiency (serum creatinine >1.5 mg/dl)
Diabetes mellitus
Poor renal perfusion: Intravascular volume depletion, congestive heart failure, liver failure
Multiple myeloma
High dose of contrast used
Prevention
Use of less nephrotoxic contrast agents (such as low-osmolal or iso-osmolal contrast
agents)
Avoid volume depletion and nonsteroidal anti-inflammatory drugs
Expansion of the intravascular space and enhanced diuresis with IV bicarbonate (bolus of
3 mL/kg of isotonic bicarbonate for 1 hour prior to the procedure, followed by 1 mL/kg/h
for 6 hours after the procedure) or normal saline (1 mL/kg/hour, 6 to 12 hours prior to the
procedure, and continued 6 to 12 hours after contrast administration)
N-acetylcysteine 1,200 mg orally twice daily the day before and the day of the procedure
Prophylactic renal replacement therapies for the prevention of contrast-induced
nephropathy have no proven benefit
Trial of pharmacologic agents, including furosemide, fenoldopam, dopamine, mannitol,
calcium channel blockers, have failed to show benefit
Pigment Nephropathy
Acute tubular injury can occur from release of non-protein heme pigment released from
myoglobin or hemoglobin
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Clinical Presentation
Acute renal failure after starting a new medication (the onset ranges from days to weeks
after initiation of the drug)
The classic signs of drug-induced AIN includes: Fever, macular rash, and eosinophilia
Dipstick evaluation is positive for proteinuria and hematuria
Urinary sediment shows RBCs (RBC casts or dysmorphic RBCs are usually not seen),
WBC and WBC casts
The presence of eosinophiluria suggests AIN, but urine eosinophils may also be seen with
acute glomerulonephritis, acute pyelonephritis, and atheroembolic renal disease
Mild proteinuria is seen (nephrotic-range proteinuria can be induced by NSAIDs when
there is a coexistant glomerular lesion which resembles minimal change disease)
Oliguria is often present (which may be related to severe interstitial inflammation causing
tubular obstruction and impeding the urine flow)
Tubular defects (Fanconi syndrome, renal tubular acidosis) are rarely seen in AIN
Renal biopsy is often required to establish a definitive diagnosis of AIN
The hallmark of AIN is the infiltration of the interstitial space by inflammatory cells (mostly
T-cells and monocytes) with sparing of glomeruli
Treatment
Discontinuation of offending agent
Patients who do not show improvement of renal function after discontinuation of the
offending drug may be treated with corticosteroids; however, their role in the treatment
of AIN remains controversial
Atheroembolic Renal Disease
AKI results from cholesterol emboli, which dislodge in medium or small renal arteries
Occurs spontaneously or following a catheter manipulation in aorta or surgery
Clinical manifestations usually appear 1 to 14 days after the inciting event, but the onset
can be delayed for weeks
Skin manifestations include livedo reticularis, purple toes, skin ulcerations and nodules
Other extrarenal manifestations include abdominal pain, pancreatitis, transient cerebral
ischemia
Renal impairment is usually subacute and advances in a stepwise fashion over a period of
several weeks, leading to need for dialysis in up to 40% of patients
Laboratory findings include increased ESR, leukocytosis and anemia, eosinophilia,
eosinophiluria, and hypocomplementemia
Treatment: Supportive care only; prognosis is poor (only half of the patients needing
dialysis regain sufficient renal function to come off dialysis)
Postrenal Azotemia
Results from obstruction of the ureters (calculi, infiltration of the ureteric wall by
malignancy, or external ureteral obstruction due to retroperitoneal fibrosis), from
obstruction of the bladder neck (prostatic hypertrophy, neurogenic bladder, or due to
treatment with anticholinergic agents), or less commonly from acute lower urinary tract
obstruction (urethral spasm, calculi or blood clots)
If obstruction is complete, anuria will result
Partial obstruction may result in polyuria due to loss of tubular function or excretion of
retained solutes
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Table 9.9: Diagnosis of HRS Criteria
1. Cirrhosis with ascites
2. Serum creatinine >1.5 mg/dL
3. No improvement of serum creatinine (decrease to a level of 1.5 mg/dL) after at least 2 days with
diuretic withdrawal and volume expansion with albumin. The recommended dose of albumin is 1 g/kg
of body weight daily up to a maximum of 100 g/d
4. Absence of shock
5. No current or recent treatment with nephrotoxic drugs
6. Absence of parenchymal kidney disease, as indicated by proteinuria >500 mg/24 h, microhematuria
(>50 RBC/hpf), and/or abnormal renal ultrasonography
Treatment
Given the dismal prognosis of patients with type 1 HRS, aggressive treatment is indicated
only in patients who are waiting for a liver transplant or for patients undergoing
evaluation to determine candidacy for transplantation
Several systemic and splanchnic vasoconstrictors have been used in HRS, including
vasopressin, vasopressin analogues (ornipressin and terlipressin), somatostatin analogue
(octreotide), and alpha-adrenergic agonists (midodrine and norepinephrine)
Transjugular intrahepatic portosystemic shunt (TIPS) has been shown to improve the
renal function of patients who receive this treatment because of refractory ascites,
variceal bleeding, refractory hepatic hydrothorax, and pre-surgical portal decompression
Renal replacement therapy can be considered as a bridge to liver transplantation
Liver transplantation is the best treatment for suitable patients with HRS because it cures
the diseased liver and the renal dysfunction
Tumor Lysis Syndrome (TLS)
A constellation of metabolic abnormalities (hyperuricemia, hyperkalemia,
hyperphosphatemia, and hypocalcemia) that develops after administration of
cytotoxic chemotherapy or occasionally occurs spontaneously in patients with tumors
characterized by high cellular proliferation and rapid cellular turnover
More commonly seen in hematologic malignancies, such as non-Hodgkin lymphoma
(in particular Burkitt lymphoma), acute lymphoblastic leukemia (ALL), acute myeloid
leukemia (AML)
Can also develop in solid tumors, such as small cell lung cancer, germ cell tumors, and
melanoma
Acute kidney injury results from hyperuricemia and hyperphosphatemia, causing tubular
obstruction with uric acid and calcium phosphate crystals. Uric acid may also activate
proinflammatory cells (neutrophils and macrophages), and may directly damage renal
tubular epithelial cells
Treatment of TLS is focused on preventing clinical complications, including AKI
Volume expansion with crystalloid solutions and forced diuresis are used to promote
urine flow and excretion of uric acid, potassium, and phosphorus
Urine alkalinization can increase the solubility of uric acid, but does not increase xanthine
solubility (a purine metabolite that can also precipitate in the renal tubules), may increase
calcium-phosphate crystallization, and may result in metabolic alkalosis
Drugs that lower uric acid levels include allopurinol and rasburicase
Purine metabolites of nucleic acids are broken down by xanthine oxidase to
hypoxanthine, xanthine, and finally uric acid. Allopurinol inhibits xanthine oxidase, thus
decreasing uric acid production. Therefore, allopurinol is more effective at preventing
hyperuricemia than decreasing preexistent hyperuricemia
Rasburicase is recombinant urate oxidase that can convert uric acid to a more soluble
metabolite, allantoin. Rasburicase is contraindicated in pregnancy and in patients with
glucose-6-phosphate dehydrogenase (G6PD) deficiency
Renal replacement therapy may be indicated in patients with refractory, life-threatening
electrolytes abnormalities, especially in the presence of volume overload and renal failure
HIV Infection Treated with HAART
Rhabdomyolysis can be seen in patients treated with zidovudine or didanosine
Tenofovir, abacavir, didanosine, lamivudine, and stavudine have been linked to the
development of proximal tubular dysfunction, including Fanconi syndrome
Acute interstitial nephritis has been described in patients taking indinavir, abacavir,
ritonavir, and atazanavir
Urinary obstruction can develop secondary to stones associated with drugs, such as
indinavir
Renal function should be monitored closely in all patients with HIV receiving any
antiretroviral agent
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9.8 Kidney Stones
Epidemiology
Kidney stones are common in industrialized countries, with an annual incidence of over 1 in
1,000 persons
Stone formation is more common in males than females (at a ratio of 2-4:1) and is more
common in Caucasian population
The peak incidence occurs in the 3rd decade of life
Renal stones generally consist of calcium salts (>70%), uric acid, cystine, or struvite
Recurrent rates are high: 40 to 50% by 5 years, and 50 to 60% by 10 years
Calcium Stones
Calcium oxalate and calcium phosphate stones are the most common stones seen in clinical
practice
Several risk factors have been described in patients with calcium containing stones:
Hypercalciuria
The most common metabolic abnormality described in patients with calcium-containing
stones, occurring in 50 to 70% of all patients with calcium stones
See Table for Causes of Hypercalciuria
Patients with idiopathic hypercalciuria have enhanced intestinal calcium absorption, and
may also have decreased renal tubular reabsorption, and increased calcium mobilization
from bone
Prevention is directed toward increasing the solubility of calcium in the urine:
High fluid intake (2-2.5L/d) to reduce the calcium concentration
Dietary sodium restriction reduces urine calcium excretion
Dietary protein restriction
Moderation of calcium intake (calcium binds to gastrointestinal oxalate and thus
prevents oxalate absorption)
Thiazide diuretics stimulate distal nephron calcium reabsorption and are the first-line
therapy for idiopathic hypercalciuria. Diuretic-induced hypokalemia can predispose
to hypocitraturia, a risk factor to stone pathogenesis. If hypokalemia develops,
potassium citrate supplements (Urocit K 20 to 40 mmol daily) can be given
Cystine Stones
Cystinuria is an autosomal recessive disorder characterized by impaired transport of cystine
and the dibasic amino acids ornithine, lysine, and arginine in the renal proximal tubules and
gastrointestinal tract, of which cystine is the least soluble and therefore the most likely to
precipitate as a stone
Patients with cystinuria start developing stones during the 1st to 3rd decade
Urine alkalinization with potassium citrate is indicated to increase cystine solubility
Protein restriction will also decrease cystine load
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Chelating drugs, such as penicillamine and mercaptopropionylglycine (tiopronin), solubilize
cystine by forming disulfide bonds with cystine
Clinical Manifestations
Include the following: Asymptomatic microscopic hematuria, renal colic (sudden onset of
flank pain, associated with nausea, vomiting, or urinary frequency and urgency), recurrent
urinary tract infection, or obstructive uropathy
Stones <5 mm in diameter will generally pass with hydration alone, but passage of stones
greater than 7 mm in diameter is uncommon and requires urologic consultation
Laboratory Studies
All patients with nephrolithiasis should undergo a basic evaluation, including serum
chemistry analysis, calcium, uric acid level, intact parathyroid hormone level (if calcium level
is high or high normal), urinalysis with sediment examination (e.g., presence of hexagonal
crystals of cystinuria), and urine culture to exclude infection with a urea-splitting organism
Patients with recurrent stones or multiple stones on 1st presentation, or patients with
non-calcium-based stones, require a more complete evaluation to evaluate for metabolic
abnormalities that can contribute to stone formation. 24-hour urine studies should be
obtained for calcium, uric acid, citrate, sodium, and oxalate excretion. A 24-hour urine
creatinine clearance collection should also be performed to ensure the adequacy of
collection
Imaging Studies
A plain film of the abdomen may reveal only radiopaque stones
Renal ultrasound may not reveal small stones or stones in the ureters and urethra
Intravenous pyelography has a high sensitivity and specificity in the diagnosis of renal
stones, but requires intravenous iodinated contrast agents
Noncontrast helical CT of the abdomen and pelvis has become the gold standard test for
diagnosis kidney stones because of its superior sensitivity and specificity in identifying
ureteric stones
Renal Hemodynamics
Normal pregnancy is characterized by widespread vasodilation, with increased arterial
compliance and reduced systemic vascular resistance. These hemodynamic changes are
accompanied by increases in renal blood flow and glomerular filtration rate
The physiologic increase in GFR is associated with a decrease in serum creatinine
concentration, which falls by an average of 0.4-0.5 mg/dL
Because the rise in renal plasma flow is slightly more pronounced than the increase in GFR,
filtration fraction decreases slightly during pregnancy
In response to renal vasodilation, there is stimulation of all components of the renin-
angiotensin system. The increased renin release causes an increase in aldosterone
production, with sodium retention and a slight decrease in serum potassium level
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Minute ventilation rises in early pregnancy and continues to increase until term, resulting in
a modest fall in the PCO2 and mild respiratory alkalosis. These changes are probably the
result of direct stimulation of the central respiratory centers by progesterone. The metabolic
compensation results in an increase in renal bicarbonate excretion, with a decrease in
plasma bicarbonate concentration by approximately 4 mmol/L
There is a small reduction in serum anion gap during pregnancy
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Gestational Hypertension
Defined as de novo hypertension developing in the latter half of pregnancy not
associated with the systemic features of preeclampsia (e.g., proteinuria), returning to
normal within 3 months postpartum
May be early manifestation of preeclampsia or early unrecognized chronic hypertension
Approximately 15 to 45% of women diagnosed with gestational hypertension develop
preeclampsia
Progression to preeclampsia is more likely to occur if gestational hypertension appears
early in the pregnancy, or if there is a history of prior miscarriage or hypertension during
a previous pregnancy
Baseline urinalysis and blood chemistries are indicated to rule out preeclampsia
Chronic Hypertension
Present in up to 5% of pregnancies
Risk of superimposed preeclampsia is 10 to 25% compared with 5% of normotensive
pregnancies
Patients with mild essential hypertension (systolic 140 to 159 or diastolic 90 to 99 mmHg)
without evidence of target organ damage may have their antihypertensive drugs tapered
during pregnancy, since blood pressure usually decreases as pregnancy progresses, with
close monitoring of the maternal blood pressure response
A subgroup of women with mild hypertension and associated comorbidities (such as
chronic kidney disease, diabetes mellitus) or in the presence of target-organ damage
(e.g., microalbuminuria, left ventricular hypertrophy) are at greater risk of maternal or
fetal complications and may benefit from antihypertensive therapy
Severe hypertension (blood pressure >160/105 mmHg), should be treated to protect the
mother from serious complications, such as stroke, heart failure, or renal failure
Diabetes Mellitus
Diabetic women with microalbuminuria have an increase in urinary protein excretion, and up
to 30% of patients may develop nephrotic range proteinuria. Postpartum, urinary albumin
excretion typically returns to pre-pregnancy values
Patients with normal blood pressure and preserved renal function have a good outcome,
but they have an increased risk of preeclampsia and urinary tract infection
In patients with diabetic nephropathy and preserved kidney function, deterioration of renal
function during pregnancy is uncommon
Patients with overt nephropathy and impaired renal function have a high incidence of
premature delivery and deterioration in renal function
Tight glucose control is associated with improved fetal outcome. Women taking
angiotensin-converting enzyme inhibitors or angiotensin receptor blockers should be
switched to other agents before conception
Chronic Glomerulonephritis
In general, pregnancy in patients with preserved renal function and normal blood pressure
has a good prognosis
Patients with scleroderma and periarteritis nodosa are at risk of increased maternal death
and poor fetal outcomes; thus, they should be counseled to avoid pregnancy
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Polycystic Kidney Disease
Pregnancy in women with autosomal-dominant polycystic kidney disease is uneventful if
renal function is preserved
Patients with hypertension and impaired renal function are at risk for preeclampsia and
premature delivery
Chronic Pyelonephritis
Bacteriuria in pregnancy may lead to acute exacerbations but otherwise is well tolerated
Frequent urine cultures are usually recommended
Chronic suppressive antibiotic therapy may be necessary
Thrombotic Microangiopathy
Acute kidney injury associated with microangiopathy and thrombocytopenia can be caused
by thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) or
severe preeclampsia with the HELLP syndrome
TTP associated with ADAMTS13 deficiency occurs predominantly in the 2nd and 3rd
trimesters
Pregnancy-associated HUS occurs more commonly in the postpartum period. Mutations
in genes that encode proteins involved in complement alternative pathway have been
associated with pregnancy-associated HUS
Treatment of TTP/HUS includes plasma infusion/exchange and other modalities used in
non-pregnant patients
Clinical Manifestations
Usually asymptomatic until the late stages of renal failure
Are nonspecific, can mimic many other clinical conditions, and include the following:
Anorexia, nausea, vomiting, and dysgeusia
Fatigue, weakness, lethargy
Pruritus
Insomnia, irritability, paresthesias, mental status changes due to encephalopathy
Dyspnea and peripheral edema (from fluid overload), chest pain from pericarditis
Physical examination findings:
Uremic fetor (foul-smelling breath similar to urine or fish)
Pallor
Signs of volume overload
Pericardial friction rub
Asterixis
Metabolic abnormalities are often seen
Anemia
Metabolic acidosis
Hyperkalemia
Hypocalcemia, hyperphosphatemia, and renal bone disease
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General Management
Early referral to nephrologist has been shown to improve outcomes and allows for early
intervention
Management of patients with CKD involves the following issues:
Prevention or Slowing the Progression of Renal Disease
ACE inhibitors and angiotensin receptor blockers (ARBs) decrease intraglomerular
pressure and hyperfiltration; may be associated with hyperkalemia
Management of hypertension
2011 KDIGO clinical guideline recommendations for management of blood pressure in
CKD patients depending upon the degree of proteinuria
Blood pressure <130/80 mmHg in patients with proteinuric CKD (500 to 1,000 mg/d
or more)
Blood pressure <140/90 mmHg in patients with nonproteinuric CKD (less than 500
to 1,000 mg/d)
The proteinuria goal is less than 1,000 mg/d
Dietary protein restriction
In theory, reduced protein intake decreases intraglomerular pressure and metabolic
demands on the kidney, but its benefit in delaying progression of CKD in humans
remains controversial
Recommended protein intake: 0.8 to 1.0 g/kg per day of high biologic value protein,
with the lower value used in patients with progressive CKD
Treatment of hyperglycemia
Goal of glycated hemoglobin should be less than 7%
Modify other cardiovascular risk factors (hyperlipidemia, hyperuricemia, tobacco use)
Prevention/Treatment of the Complications of CKD (Volume Overload, Anemia,
Hyperkalemia, Metabolic Acidosis, Renal Bone Disease)
Volume Overload
Results from the inability of kidney to excrete water and salt
Treatment includes dietary sodium restriction and diuretic therapy, usually with a loop
diuretic given daily
Anemia
Anemia of chronic kidney disease is normocytic and normochromic, and results from
decreased production of erythropoietin by the kidney, and to shortened red cell survival
Anemia is common in patients with CKD with GFR <60 mL/min
Anemia is a contributing factor in many of the symptoms associated with reduced kidney
function. These include fatigue, decreased functional capacity and quality of life, dyspnea,
and cardiovascular consequences, such as left ventricular hypertrophy
The evaluation of anemia in patients with CKD should begin when the Hgb level is less
than 12 g/dL in females, and less than 13.5 g/dL in adult males
The evaluation of anemia should rule out other causes, and includes reticulocyte count,
serum iron, total iron binding capacity, percent transferring saturation, serum ferritin,
peripheral smear, and Hemoccult stool
Treatment of anemia includes the use of erythropoiesis-stimulating agents (ESAs)
ESAs should be initiated when the Hgb level is <10 g/dL; goal maintaining Hgb levels
between 10 and 11 g/dL
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Clinical manifestations of renal osteodystrophy
Bone pain and fractures
Muscles weakness and myopathy
Spontaneous tendon rupture
Pruritus (associated with severe hyperparathyroidism, and a high calcium-phosphorus
product)
Metastatic and extraskeletal calcifications
Renal Bone Disease
The initial step in the treatment of renal bone disease should include dietary phosphate
restriction
An intake of approximately 800 mg/d is recommended by the K/DOQI guidelines
The K/DOQI guidelines recommend that serum phosphorus levels should be between 2.7
and 4.6 mg/dL among patients with stage 3 and 4 CKD, and between 3.5 and 5.5 mg/dL
among those with stage 5 CKD
Many patients require phosphate binders to reduce the amount of phosphorus absorbed
and achieve a normal phosphate concentration
Calcium-based binders: Calcium carbonate, calcium acetate
Non-calcium-based binders: Sevelamer hydrochloride, lanthanum carbonate
Aluminum-based binders should be avoided because of the gradual induction of
aluminum toxicity
If the target PTH level is not achieved, despite dietary phosphate restriction and intestinal
phosphate binders, administration of vitamin D compounds is recommended:
1,25-OH-vitamin D (calcitriol), or
Synthetic vitamin D analogues (paricalcitol or doxercalciferol)
Vitamin D analogs can induce hypercalcemia and hyperphosphatemia and therefore
should be used with caution
The goal iPTH levels varies, based on stage of CKD
Stage III: iPTH = 35-70
Stage IV: iPTH = 70-110
Stage V: iPTH = 150-300
Calcimimetics are agents that allosterically increase the sensitivity of the calcium-
sensing receptor in the parathyroid gland to calcium. Cinacalcet is currently the only
available calcimimetic, is curently approved only for dialysis patients, but may become
an emerging option in the treatment of secondary hyperparathyroidism in predialysis
patients with CKD
Preparation of the Patient for Renal Replacement Therapy
The 2006 K/DOQI guidelines recommend that patients with a GFR less than 30 mL/min
should be educated concerning different choices of renal replacement therapy, such as
hemodialysis (in-center or at home), peritoneal dialysis, and renal transplantation (living or
deceased donor)
Renal Transplantation
Kidney transplantation is the treatment of choice for end-stage renal disease
Patients can be listed for cadaveric transplant when eGFR is less than 20 mL/min
Contraindications to transplantation include: Recent or metastatic malignancy, current
untreated infection, history of nonadherence, inability to give informed consent, and
active use of illicit drugs
Graft survival rates exceeds 95% at 1 year and 80% at 5 years
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The endothelial cells are attached to the glomerular basement membrane (GBM),
composed of a complex meshwork of different matrix proteins, such as type IV
collagen, laminin, fibronectin, polyanionic proteoglycans (mostly heparan sulfate), and
other glycoproteins
Visceral epithelial cells (or podocytes) are structurally complex cells that possess
interdigitating processes (foot processes), which are attached to proteoglycans in
the GBM via adhesion proteins (integrins). The pores between the foot processes (slit
pores) are closed by a thin membrane called the slit diaphragm. A number of proteins
have been found to be part of the slit diaphragms, including nephrin, neph1, neph2,
FAT1, FAT 2, podocin, TRPC6, and tight junction proteins
Mesangium composed of the mesangial matrix (similar in composition to the GBM but
less well organized) and the mesangial cells, which are contractile, phagocytic cells,
capable of proliferation, of laying down matrix and collagen, and of secreting many
biologically active mediators
Injury to the visceral epithelial cells (e.g., foot process effacement or detachment of
podocytes from the underlying GBM) or injury to the GBM (such as thickening of the GBM
due to increased synthesis of its protein components in diabetic glomerulosclerosis, or due
to deposition of amorphous electron-dense material, most often immune complexes) can
alter the permeability of the capillary wall, and results in proteinuria
Rupture of the capillary wall or cellular proliferation of the mesangial or endothelial cells
causes hematuria
Rupture of the GBM following an immune/inflammatory injury can result in necrosis and
crescent formation. These are accumulations of cells composed of proliferating parietal
epithelial cells and infiltrating leukocytes
Clinical Manifestations
Majority of patients who develop glomerular disease present with 1 of 2 patterns of
kidney disease, nephritic or nephrotic, based upon the urine sediment and the degree of
proteinuria
Nephritic Pattern
Nephritic disorders are commonly associated with systemic diseases and are often
caused by immune-complex deposition in the glomerular capillaries
Usually characterized by azotemia, oliguria, hypertension, proteinuria (ranging from
normal protein excretion to the nephrotic range) and active urine sediment characterized
by the presence of red cells (some with a dysmorphic appearance and acanthocytes) and
occasionally white blood cells, with or without red cell or mixed cellular casts, and variable
degrees of proteinuria, ranging from normal protein excretion to the nephrotic range (i.e.,
both nephritic and nephrotic)
Common glomerular diseases presenting as nephritic syndrome include:
Poststreptococcal glomerulonephritis, other postinfectious diseases (endocarditis,
shunt nephritis, abscesses), IgA nephropathy, systemic lupus erythematosus, vasculitis,
Goodpastures disease
Serologic work-up include antistreptococcal antibodies, antinuclear antibodies, anti
double-stranded DNA antibodies, ANCA, antibodies, anti-GBM antibodies, cryoglobulins,
hepatitis B or C virus antibodies, and complement levels
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Immunosuppressive agents (cyclophosphamide, cyclosporine, tacrolimus, and
mycophenolate mofetil) have been used in patients with frequent relapses, as well as
in corticosteroid-resistant patients
Focal Segmental Glomerulosclerosis (FSGS)
Accounts for 30 to 35% of cases of adult nephrotic syndrome
The most common primary glomerular disease that causes end-stage kidney disease in
the US
The yearly incidence of primary FSGS has increased over the past 2 decades
Idiopathic FSGS is more prevalent among African American and Hispanic population
FSGS may be primary (idiopathic) or may be associated with other conditions:
Reduced nephron numbers (unilateral renal agenesis, postnephrectomy, reflux
interstitial nephritis) thought to represent an adaptive response to glomerular
hypertrophy or hyperfiltration
HIV disease (usually collapsing variant)
Toxins (heroin, interferon, lithium, and pamidronate)
Glomerulomegaly (morbid obesity, cyanotic congenital heart disease, or hypoxic
pulmonary disease)
Familial FSGS described in patients with mutations involving various slit diaphragm
proteins (alpha-actinin 4, podocin, nephrin, or TRPC6)
The pathogenesis of primary FSGS involves injury to the visceral epithelial cell or
podocyte. Injury may be caused by a circulating toxin, such as the soluble urokinase
receptor (suPAR)
Pathology
LM: Segmental areas of mesangial collapse and sclerosis in some but not all glomeruli
(focal); sclerotic changes occur first in juxtamedullary glomeruli
IF: No true immune complex deposits, except for nonspecific binding of IgM and C3
in the mesangium associated with sclerotic lesions
EM: Diffuse fusion of the epithelial cell foot processes
In addition to classic FSGS, variants include collapsing, tip, perihilar, and cellular.
Collapsing FSGS is usually but not always associated with HIV infection, and it is
characterized by collapse and sclerosis of the entire glomerular tuft, associated often with
severe tubular injury with proliferative microcyst formation and tubular degeneration.
The collapsing variant has a worse prognosis than other variants. The tip variant has the
defining pathologic lesion located at the tip of the glomerulus near the proximal tubule,
and is considered the most steroid responsive variant
Clinical manifestations
Proteinuria is the hallmark of primary FSGS
Patients with FSGS also present with microscopic hematuria, hypertension, and renal
failure
Predictors of poor outcome in primary FSGS include: Nephrotic range proteinuria, renal
failure, and failure to respond to corticosteroid treatment
Treatment
Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers
(ARBs) are the principal treatment agents used for non-nephrotic patients
Nephrotic patients with primary FSGS should receive immunosuppressive therapy
with corticosteroids
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Resistant patients (patients with moderate or high risk disease who fail an adequate
trial of treatment with both cyclophosphamide-based and calcineurin inhibitor-based
regimens) can be given a trial of rituximab
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The pathogenesis of HIVAN is not well understood, but may involve direct viral infection
of glomerular endothelial, mesangial and tubular cells
Patients with HIVAN present with nephrotic syndrome, renal failure and rapid progression
to end-stage renal disease
The renal ultrasound shows large echogenic kidneys
Therapies that could slow the progression of HIVAN include HAART therapy and
angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARB). The
role of corticosteroids, cyclosporine or other immunosuppressants in HIVAN is uncertain
Other renal conditions associated with HIV infection include:
Membranous nephropathy, associated with concurrent infection with hepatitis B and
C infection or syphilis
Membranoproliferative glomerulonephritis, associated with concurrent hepatitis C
infection or mixed cryoglobulinemia
IgA nephropathy
A lupus-like, immune complex mediated glomerulonephritis
Interstitial nephritis, due cytomegalovirus infection or a reaction to a medication (e.g.,
TMP-SMX)
Amyloidosis due to chronic infection
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)
Nephritic Syndromes
IgA Nephropathy
IgA nephropathy is the most common cause of glomerulonephritis in most countries
IgA nephropathy is common in Asian and Caucasian population, and is rare in African
decent patients
Pathology
LM: Diffuse mesangial proliferation and matrix expansion with proliferative
glomerulonephritis and crescents in more severe disease
IF: Prominent, globular deposits of IgA (often accompanied by C3 and IgG) in the
mesangium
EM: Electron-dense deposits located predominantly in the mesangium
Clinical manifestations of IgA nephropathy are limited to the kidney. Mesangial IgA
deposition, which is often clinically silent, may also be associated with cirrhosis, celiac
disease, ankylosing spondylitis, or HIV infection. In addition, IgA nephropathy may also
be seen with other glomerular diseases, such as minimal change disease and Wegeners
granulomatosis
Clinical manifestations
Gross hematuria, usually following an upper respiratory or gastrointestinal infection
Microscopic hematuria with or without proteinuria
Rarely, patients with IgA nephropathy can develop acute kidney injury with or
without oliguria, due to crescentic IgA nephropathy, or to gross hematuria causing
tubular occlusion by red cells
Treatment
Nonimmunosuppressive therapies with angiotensin converting enzyme (ACE)
inhibitors or angiotensin II receptor blockers (ARBs), and fish oil are indicated for
patients with isolated hematuria, minimal proteinuria (less than 1,000 mg/d) and
normal kidney function
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Lupus Nephritis (LN)
40 to 50% of patients with SLE develop renal abnormalities early in their course
Clinical manifestations include microhematuria, proteinuria, hypertension, and progressive
renal dysfunction
The ISN classification system divides glomerular disorders associated with SLE into 6
different classes based on kidney biopsy histopathology:
Class I: Minimal mesangial LN
Normal glomeruli by LM, but mesangial immune deposits by IF
Class II: Mesangial proliferative LN
Mesangial hypercellularity with mesangial immune deposits
Class III: Focal LN
III (A): Purely active lesions: Focal proliferative LN
III (A/C): Active and chronic lesions: Focal proliferative and sclerosing LN
III (C): Chronic inactive lesions with glomerular scars: Focal sclerosing LN
Class IV: Diffuse LN
IV-S (A) or IV-G (A): Purely active lesions: Diffuse segmental (S) or global (G)
proliferative LN
IV-S (C) or IV-G (C): Inactive with glomerular scars: Diffuse segmental of global
sclerosing LN
Class V: Membranous LN
Class VI: Advanced sclerosing LN
90% of glomeruli globally sclerosed without residual activity
Treatment
Class I and II disease is associated with excellent prognosis, and immunosuppressive
therapy in these patients is not indicated
The patients with active focal proliferative LP (ISN class IIIA and III A/C), active
diffuse proliferative LN (ISN class IV A and IV A/C), and membranous lupus (ISN
class V) are usually treated with immunosuppressive therapy
Anti-glomerular Basement Membrane Antibody Disease
Anti-GBM antibody disease is caused by antibodies to a noncollagenous (NC1) domain of
type IV collagen, which is highly expressed in the GBM and alveoli
The pulmonary-kidney presentation of anti-GBM antibody disease, known as Goodpasture
syndrome, causes pulmonary hemorrhage with rapidly progressive glomerulonephritis
40% of patients with anti-GBM antibody disease also have ANCA-associated vasculitis
Pathology
LM: Crescentic glomerulonephritis
IF: Linear deposition of IgG along the glomerular capillaries and occasionally the distal
tubules
Treatment includes plasmapheresis in conjunction with cyclophosphamide and
prednisone
Small-vessel Vasculitis
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides include
granulomatosis with polyangiitis (Wegeners), which can be abbreviated as GPA,
microscopic polyangiitis (MPA), the Churg-Strauss syndrome (CSS) and renal limited
vasculitis
SUGGESTED READINGs
1. Bagshaw SM, Haase M, Hasse-Fielitz A, Bennett M, Devarajan P, Bellomo R. A Prospective
Evaluation of Urine Microscopy in Septic and Non-septic Acute Kidney Injury. Nephrology Dialysis
Transplantation. Vol 27(2), p582-588.
2. Bevc S, Hojs R, Ekart R, Zavrsnik M, Gorenjak M, Puklavec L. Simple Cystatin C Forumla for
Estimation of Glomerular Filtration Rate in Overweight Patients with Diabetes Mellitus Type 2 and
Chronic Kidney Disease. Experimental Diabetes Research. Vol 2012.
3. Molitoris BA. Measuring Glomerular Filtration Rate in Acute Kidney Injury: Yes, But Not Yet. Critical
Care. 2012, 16:158.
4. Daugirdas JT, Leypoldt JK, Akonur A, Greene T, Depner TA, the FHN Trial Group. Improved Equation
for Estimating Single-pool Kt/V at Higher Dialysis Fequencies. Nephrology Dialysis Transplantation.
2012.
5. Reidenberg MM. Early Research on Renal Function and Drug Action. The Journal of Clinical
Pharmacology. 2011.
6. Kovesdy CP. Significance of Hypo- and Hypernatremia in Chronic Kidney Disease. Nephrology
Dialysis Transplantation. Vol 27(3):891-898.
7. Atta MG. Dilutional Hyponatremia in Cirrhosis: Updating the Standard of Care. Nephrology Times.
April 2012, 5(4), p12-14.
8. Franco MCP, Oliveira V, Ponzio B, Rangel M, Palomino Z, Gil FZ. Influence of Birth Weight on
the Renal Development and Kidney Diseases in Adulthood: Experimental and Clinical Evidence.
International Journal of Nephrology. Vol 2012.
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9. Thomas W, Griffiths M, Nelson-Piercy C, Sinnamon K. Pre-eclampsia Before 20-week Gestation:
Diagnosis, Investifation and Management. Clinical Kidney Journal. Oct 2012.
10. Bushinsky DA, Wolf MS. Mineral Metabolism: Confusion to Clarity. Current Opinion in Nephrology &
Hypertension. Jul 2012, Vol 21(4), p353-354.
11. Bushinsky DA. Clinical Application of Calcium Modeling in Patients With Chronic Kidney Disease.
Nephrol Dial Transplant (2012) 27:10-13.
12. Riggs MM, Peterson MC, Gastonguay MR. Multiscale Physiology-based Modeling of Mineral Bone
Disorder in Patients With Impaired Kidney Function. The Journal of Clinical Pharmacology. 2012.
13. Dash A, Fatima H, Grewal M, Galphin C, Paueksakon P. Renal Injury Due to Anti-glomerula Basement
Membrane Antibody-mediated Glomerulonephritis Without Circulation Antibody. Clin Kidney J
(2012) 0:1-4.
14. Oda T, Yoshizawa N, Yamakami K, Sakurai Y, Takechi H, Yamamoto K, Oshima N, Kumagai H. The
Role of Nephritis-associated Plasmin Receptor in Glomerulonephritis Associated With Streptococcal
Infection. Journal of Biomedicine and Biotechnology. 2012.
15. Shah S, Weber-Shrikant E, Panesar M. Discontinuation of Antiretroviral Therapy Causing Progression
to End-stage Renal Disease in an HIV Patient Diagnosed With Immune Complex 'Lupus-like'
Glomerulonephritis. Clinical Kidney Journal. Vol 5(3) p276-278.
16. Villaverde RV, Darioli V, Hirschel B, McKee TA, Lobrinus JA, Moll S. Kidney Light Chain Disease in
Patients With the Acquired Immunodeficiency Syndrome. Clinical Kidney Journal. Vol 5(1)p59-62.
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NOTES
Wasseem El-Aneed, MD
C o n t e n t s
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10.1 INTRODUCTION
The hematology chapter focuses on high-yield information regarding 4 important topics: Any
topics that are not covered in this chapter can be found throughout the study guide, as hematol-
ogy may be viewed as a cross-content category.
Myelodysplastic Syndromes
Group of disorders characterized by:
Ineffective blood cell production
Variable progression to acute leukemia
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Most of the patients are >60 years old
Idiopathic or related to alkylating agents, radiation, or benzene
Clinically presented with symptoms related to pancytopenia (weakness, infection, bleeding)
Macrocytic anemia is the most common presentation
Diagnosis: Macrocytosis, hypergranular WBC and blasts may be seen, PLTs are giant. Bone
marrow biopsy: Hypercellular
Note: If >20% blasts present in peripheral blood, then the diagnosis is AML
Note: Deletion of chromosome 5q or 20q has favorable prognosis
Treatment
Bone marrow transplant in young patients
Chemotherapy with demethylating agents like azacitidine and decitabine
Supportive therapy
Growth factors have been used with variable response
5q disease: Generally responds to Lenalidomide
Myeloproliferative Disorders
Definition: Clonal malignancy that results from clonal expansion of multipotent HSCs, in which
the regulation of cells proliferation is impaired. There are 4 major types:
1. Essential thrombocythemia
2. Polycythemia vera
3. Chronic myeloid leukemia (CML)
4. Myelofibrosis
Essential Thrombocythemia
PLT >600,000 with normal CBC counting
Patients are at risk of thrombosis (venous and arterial) and hemorrhage. Life expectancy
may not be affected in majority of patients
Patients are likely in the 5th or 6th decade
Symptoms: Bleeding and splenomegaly
Diagnosis
Ht >60%
Mainly by excluding secondary erythrocytosis (normal to low erythropoietin)
The JAK2 mutation is present in more than 90% of the patients
Splenomegaly
Other findings will be helpful like PLT >400,000 and neutrophils >10,000
Treatment
Phlebotomy to Ht <45% found to reduce mortality though it may increase risk of
thrombosisgive ASA
Other supportive treatments include hydroxyurea (to keep PLT <400,000),
allopurinol, low dose ASA
Prognosis
Depends on leukemic transformation and thrombosis/bleeding events
Chronic Myeloid Leukemia (CML)
Definition: Myeloproliferative disorder characterized by overproduction of myeloid cells.
Present with patients >50 years old
Signs and symptoms
Fatigue, night sweats, and low-grade fever
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Treatment
Supportive: Transfusions, splenectomy, and hydroxyurea
The only curative treatment is allogeneic bone marrow transplant
Prognosis
Median survival is only 5 years. Severe anemia on presentation indicates poor
prognosis
Transfusion Reactions
Acute Hemolytic Transfusion Reaction
ABO incompatibility (medical emergency), within minutes to hours
Symptoms: Chills, backache, tachypnea, hypotension
Stop transfusion; send the bag for type and cross, vigorous hydration
Labs: Coombs, Hg, bilirubin, HDL, haptoglobin
Delayed Hemolytic Transfusion Reaction
Rh incompatibility
Happens a week after transfusion
Symptoms are mild including fever and elevated unconjugated bilirubin. No treatment is
needed
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Allergic Reaction
Causes urticaria and possible bronchospasm
Seen in IgA deficient patients (they form anti IgA-antibodies)should receive transfusion
from IgA-deficient donors
Febrile Nonhemolytic Reaction
The most common reactionnonhemolytic reaction to the transfused WBCtreat with
acetaminophen and diphenhydramine. Leukocyte depleted units can be used to minimize
this reaction
Acute Lung Injury
Respiratory distress typically 1 to 2 hours (up to 6 hours) after transfusion of FFP
Caused by donor antibodies to recipient leukocyte
Treat supportively (including intubation. Recovery is expected in 4 days)
Other Considerations
Transfusion dependant thalassemia patients develop hemosiderosis. Diagnosed with
elevated ferritinliver biopsy may be necessary. Treat with chelating agents
CMV negative blood should be given to transplant patients
GVH reaction: In immunocompromised patients, lymphocytes in donated blood attack the
host tissues
Thrombocytopenia in Pregnancy
Mild thrombocytopenia is generally benign and doesnt require further investigation. Usually
develops late in the term of pregnancy
Most common cause is preeclampsia, HEELP syndrome should be excluded
ITP might be difficult to diagnose and generally a Dx of exclusion
ITP should be treated with prednisone when PLT count < 50K, watch for gestational
diabetes
TTP is treated with plasmapheresis
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Table 10.1: You May Depend on the WHO Criteria for Diagnosis
(One major and one minor or three minor criteria)
Major Criteria Minor Criteria
Plasmacytoma on tissue biopsy Bone marrow clonal plasma cells 10%-30%
Bone marrow clonal plasma cells >30% M-protein less than as mentioned in major criteria
High M protein (IgG >3.5 g/dL [35 g/L], IgA >2.0 g/ Lytic bone lesions
dL [20 g/L], Bence-Jones proteinuria >1.0 g/24 h) Immunoglobulins to <50% of normal
Waldenstrom Macroglobulinemia
Bone marrow infiltrates with neoplastic B-cells that secrete monoclonal IgM
Symptoms include anemia (from bone marrow infiltrate), hyperviscosity secondary to high
levels of IgM, lymphadenopathy, splenomegaly and hepatomegaly. Organomegaly and
absence of bone lytic lesions help to differentiate it from MM
SPEP shows a spike of IGM, bone marrow biopsy confirms the diagnosis
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NOTES
Mustafa Hyder, MD
Genevieve Moyer, MD
Iba Al Wohoush, MD
Chukwuemeka Charles Ezeoke MD, LT (USN)
C o n t e n t s
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11.1 CANCER RISK
Environmental Risk Factors
Chemical carcinogens can lead to cancer either by DNA damage (genotoxic) or by a variety
of non-genotoxic mechanisms
Breast Cancer
Familial cancer syndromes associated with breast cancer:
Those associated with BRCA1/BRCA2 mutations
Li-Fraumeni syndrome
Mutation in TP53 gene
p53 protein is the guardian of the genome and mutations can lead to a wide
spectrum of neoplasms occurring at a young age
Autosomal dominant
Cancer occurs in 90% of women before 60 years
Management: Genetic counseling and testing to confirm.
Diagnosis : 3 criteria: diagnosis of sarcoma at a young age (below 45) + cancer
diagnosis in a 1st degree relative < 45 years old + cancer diagnosis in ANOTHER
1st degree relative < 45 years old
Cowden syndrome
High risk of benign fibrocystic breast disease
25 to 50% lifetime risk of breast cancer
Mutations in PTEN, tumor suppressor gene
Manifestations : predisposition to endometrial, breast, thyroid carcinoma with skin
tumors, macrocephaly, intestinal polyps, and benign skin tumors
Offspring of individual with germline mutation has 50% chance of inheriting mutation
Hereditary Colorectal Cancer Syndromes
Main high risk colorectal cancer syndromes:
Lynch syndrome (hereditary nonpolyposis colorectal cancer)
Polyposis syndromes
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Classical familial adenomatous polyposis
Attenuated familial adenomatous polyposis
Peutz-Jeghers syndrome
Juvenile polyposis
MUTYH-associated polyposis
Lynch syndrome
Most common genetically determined colon cancer risk factor
2 to 4% all CRC cases
Germline mutation in DNA mismatch repair gene
Microsatellite instability
80% lifetime risk of developing colorectal cancer (mean age of diagnosis is 44 years)
The Amsterdam criteria developed in 1990 was previously used, but was replaced
by the Bethesda criteria (developed in 1996) due to its low sensitivity in detection
(about 50%)
The Bethesda criteria is 89% sensitive, and 80 % specific , and recommends that
patients with colon cancer should be tested for microsatellite instability and should
further be tested for germline MMR
Most common extra-colonic cancer in Lynch is endometrial cancer
Classical and attenuated FAP
Autosomal dominant
Germline mutation in APC gene
Less than 1% of all CRC
>100 polyps on colonoscopy is diagnostic of classical FAP
Lifetime cancer risk approaches 100% by age 50
Attenuated typically has later onset and <100 polyps
Lifetime risk 70% by age 80
Prophylactic proctocolectomy in 2nd decade is recommended
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11.2 LUNG CANCER
Lung cancer is the most prevalent cancer and the number one cause of cancer deaths. t is
divided into small cell, oat cell, and non-small cell lung cancer. 80-90% of cases is due to tobacco
smoke; while 10-15% is from non-smoking
Clinical Presentation
Initial presentation may be based on signs, symptoms or laboratory abnormalities
Signs and symptoms
Cough, weight loss, dyspnea, chest pain, hemoptysis, bone pain, clubbing, fever,
weakness, superior vena cava obstruction, dysphagia, wheezing, stridor
Horners syndrome: Ptosis, myosis, anhydrosis
Pancoast or superior sulcus tumor: Local extension of a tumor growing in the apex
of the lung with involvement of the 8th cervical and 1st and 2nd thoracic nerves
with shoulder pain, and destruction of 1st and 2nd ribs
Paraneoplastic Eaton-Lambert: Proximal muscle weakness, autonomic dysfunction,
cranial nerve, or bulbar symptoms
Strength improves with serial effort
Trousseaus syndrome: Migratory venous thrombophlebitis
1/3 present with symptoms of distant metastases
Laboratory abnormalities
Anemia, elevated alkaline phosphatase, elevated transaminases, hypercalcemia
12% present with paraneoplastic endocrinopathies
Hypercalcemia from ectopic production of PTH or PTH-related peptide
(Squamous cell)
Hyponatremia from SIADH or atrial natriuretic peptide secretion In small cell
lung carcinoma
Treated with demeclocycline if unresponsive to fluid restriction
Hypokalemia from ectopic ACTH secretion (small cell, carcinoid)
Staging
Table 11.3: Staging
T N M
x Cannot be assessed Can not be assessed Can not be assessed
0 No evidence of primary No nodes No metastasis
Tis Carcinoma in situ
1 <3 cm Ipsilateral non-medias- A: Local
tinal or subcarinal B: Distant
2 3-7 cm Ipsilateral mediastinal
or subcarinal
3 >7 cm Contralateral,
supraclavicular
4 Invasion of mediastinum, heart or STAGE
great vessels, recurrent laryngeal
nerve, esophagus, vertebra
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Non-small Cell Lung Cancer
85% of lung cancers
Squamous and non-squamous (adenocarcinoma, large cell, other)
Squamous cell: more centrally-located, more common in men who are smokers, than
women
Large cell: has twice odds ratio in smoking women than in men.
Adenocarcinoma: Most common type of lung cancer and is the most frequent cell type in
non-smokers
Treatment
Varies depending on the stage, patients karnofsky performance status, and cancer subtype.
Different treatment modalities include surgery, chemotherapy, radiation therapy, palliative
care
Surgery
FEV1 >2L or
FEV1 1.5 - 2L FEV1 <1.5L
80% predicted
Yes No Yes No
Pneumonectomy Lobectomy
Pneumonectomy Lobectomy
Further physiologic
testing
Chemotherapy
Stage IB, II and III: In patients with completely resected NSCLC, adjuvant chemotherapy
has been shown to improve survival
Stage III: Concurrent chemoradiation is superior to sequential therapy
Stage IV: Platinum-based chemotherapy as palliation
Targeted therapies
Bevacizumab: Anti-VEGF
Erlotinib: EGFR inhibitor
Crizotinib: ALK inhibitor
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Diagnosis
Men with prostatic nodules, indurations, or asymmetry should be referred to a urologist
or abnormally elevated PSA
Transrectal ultrasound (TRUS) guided needle biopsy (minimum of 12 core samples)
The urologist should perform a Transrectal ultrasound (TRUS) guided needle biopsy
Repeat Biopsy is indicated if the first biopsy is negative, and the PSA continues to rise
Histology
95% tumors are adenocarcinomas
5% are squamous or transitional cell, rarely sarcomas
Gleason grading system
Based on dominant and secondary histology
Each receive a score from 1 (well-differentiated) to 5 (undifferentiated)
Scores are added
After confirmed biopsy, MRI is imaging modality used to complete staging
Treatment
Watchful waiting
Serial DRE, PSA, biopsies
Radical Prostatectomy
Patients with life expectancy >10 years
Commonly complicated by impotence and/or incontinence
Radiation
External beam
Brachytherapy: Implantation of radioactive seeds into prostate
Medications
Testosterone-lowering agents: Leuprolide, goserelin, degarelix
Used in metastatic disease in individuals with noncastrate levels of testosterone
(>150 ng/dL)
Associated with hot flushes, impotence, depression, insulin resistance, obesity,
increased cardiovascular disease risk and osteoporosis
Assess fracture risk with FRAX scale, supplement with calcium and vitamin D,
treatment with bisphosphonate or denosumab
Antiandrogens: Flutamide, bicalutamide, nilutamide
Primarily used to reduce side effects of testosterone-lowering agents
Testicular Cancer
Most commonly presents as painless testicular mass
Testicular mass in male >50 is lymphoma until proven otherwise
Histology
2 main histologic types: 1. Seminoma 2. Nonseminoma
Nonseminomatous further broken down into 4 histologic subtypes: Embryonal,
teratoma, choriocarcinoma, endodermal sinus (yolk sac) tumor
Embryonal: Secrete tumor markers hCG, AFP or both
Teratoma: Contains cell types from 2 or more germ layers: Ectoderm, mesoderm, or
endoderm
Choriocarcinoma: Cytotrophoblast or syncytiotrophoblast
Secrete tumor marker hCG
Endodermal sinus or yolk sac tumor
Secrete AFP
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Evaluation
Figure 11.2: Testicular Cancer Evaluation
Testicular discomfort
or swelling
Trial of antibiotics
Painless Testicular
Mass No Resolution Resolution
TESTICULAR ULTRASOUND
No further testing
MEASUREMENT OF AFP, BETA-HCG,
AND LDH CHEST X-RAY
Normal
Suspicious testicular lesion + Other abnormal findings
Elevated/rising beta hCG +
Symptomatic metastatic disease
Testicular biopsy versus orchiectomy
(malignancy on biopsy should be
followed by orchiectomy)
Chemotherapy
Bladder Cancer
Epidemiology
Up to 50% can be attributed to tobacco; up to 30% develop cancer when exposed to
benzidine
Most common form is transitional cell carcinoma
In developing countries parasitic infection with Schistosoma haematobium is a
common cause for squamous cell carcinoma of the bladder
Clinical presentation
Classically: Painless hematuria in male smoker >50 years old
Irritative symptoms such as dysuria, frequency, or urgency
Obstruction with flank pain and symptoms of metastatic disease can occur but are rare
Evaluation
Cystoscopy is imaging modality of choice
Ultrasound, CT, and/or MRI can be used to document full extent of disease
Histology
90% transitional or urothelial
3% squamous (most common in distal third of urethra)
2% adenocarcinoma
1% small cell
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Treatment
Dependent upon whether tumor is superficial or muscle-Invasive
Superficial
Endoscopic resection (transurethral resection of bladder tumor or TURBT) with
or without intravesical therapy (bacillus Calmette-Guerin instillations)
Indication for BCG therapy: Recurrent disease, >40% involvement, diffuse
carcinoma in situ
Muscle-invasive: Radical cystectomy
Removal of pelvic lymph nodes
Males: Removal of prostate, seminal vesicles, proximal urethra
Females: Removal of urethra, uterus, fallopian tubes, ovaries, anterior vaginal
wall
Requires creation of continent cutaneous reservoir or orthotopic neobladder
Consideration of chemotherapy for nodal disease, extravesical extension, or
vascular invasion
Renal Cancer
Epidemiology
Strongest association is with cigarette smoking
35% of individuals with von Hippel-Lindau (VHL) will develop RCC
80-85% develop within the renal cortex; 8% develop as transitional cell carcinoma of
the pelvis
Histology
Clinical presentation
Classic triad: Hematuria, abdominal pain, and palpable mass
Stauffer syndrome: Paraneoplastic syndrome with erythrocytosis, hypercalcemia and
hepatic dysfunction
Evaluation
Any solid renal mass should be considered malignant until proven otherwise
If no metastases, surgery is indicated
Non renal-cell malignancies of the kidney: Transitional cell carcinoma of the renal pelvis,
sarcoma, lymphoma, Wilms tumor
Radiographic testing: Abdominal ultrasound or CT is first-line test
Ultrasound is less sensitive than CT for differentiating a benign cyst from a tumor.
A cyst is all of the following: (1). No echoes within the cyst (anechoic) (2). Strong
posterior wall echo indicating good transmission through a cyst. (3). Rounded, with
demarcated smooth walls
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Brain Metastases with Increased Intracranial Pressure
Most commonly:
Lung 16 to 20 percent
Melanoma 7 percent
Renal cell cancer 7 to 10 percent
Breast cancer 5 percent
Symptoms
Headache, nausea, vomiting, behavior change, seizure
Diagnosis
Contrast-enhanced MRI is more sensitive than non-contrast or T2-weighted MRI or
contrast-enhanced CT scan
MRI with gadolinium is most sensitive in evaluating meningeal involvement and small
lesions
Treatment
Dexamethasone 6 mg IV Q6H (indicated only in patients with increased intracranial
pressure or edema on imaging)
Intubation with hyperventilation to maintain PCO2 between 25 and 30 mmHg
Mannitol 1-1.5 g/kg IV Q6H
Head elevation, fluid restriction, and hypertonic saline with diuretics
Shunt placement for patients with hydrocephalus
Whole brain radiation in patients with multiple lesions
Surgical excision for single brain metastases if extra-cranial disease is controlled
Should be followed by whole-brain radiation
Stereotactic radiosurgery for inaccessible or recurrent lesions
Spinal Cord Compression
Most common culprits are lung, breast, prostate, multiple myeloma, lymphoma,
melanoma, thyroid, renal cell, and GU cancers
Mnemonic: BLT with pickles, mustard, and relish: Breast, Lung, Thyroid, Prostate,
Myeloma/Melanoma, and Renal cell
Thoracic spine is the most common site (70%)
75% who receive treatment while still ambulatory will remain ambulatory, while only 10%
of those with paraplegia recover ambulatory ability
Symptoms
Back pain and tenderness
Radicular pain
Lhermitte sign: Tingling or electric sensation down back with neck flexion
Loss of bowel or bladder
Gait abnormalities or ataxia
Physical findings
Pain on percussion
Weakness or spasticity
Extensor plantar reflex or brisk deep tendon reflexes
Absence of anal wink
Cauda equina syndrome: Compression at level of nerve roots leaving spinal cord
Low back pain, saddle anesthesia, rectal and bladder dysfunction, sexual
impotence, absent Achilles reflex, and lower extremity weakness
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Etiology
Production of PTHrP (parathyroid hormone related protein) by solid tumor
Squamous cell carcinoma of the lung, multiple myeloma, and breast cancer are
most common culprits
The mechanisms of hypercalcemia are due to:
Production of 1, 25-dihydroxyvitamin D
Local release of cytokines secondary to osteolytic metastasis
PTH-rP secretion by the tumor cells
Findings
Elevated urinary cyclic AMP, hypophosphatemia, increased urinary phosphate
clearance
Low-normal levels of 1,25(OH)2 vitamin D
Differentiating between hyperparathyroidism and hypercalcemia of malignancy
Treatment
Hydration with normal saline until euvolemic, then hydration with loop diuretic
Bisphosphonates
Pamidronate and zoledronate
Onset of action is 1 to 2 days
Dialysis
Control of underlying malignancy
In patients with end stage malignancy, consider withholding treatment for elevated
calcium as it can provide a sedating effect
Chemotherapy-related Toxicities
Neutropenia and infection
Patients with intermediate and high risk malignancies or treatment regimens should be
on bacterial, fungal, and viral prophylaxis while neutropenic
Includes patients with liquid tumors and those with neutropenia lasting longer than
7 days
Patients at increased risk for pneumocystis jirovecci should also receive prophylactic
Bactrim, dapsone, aerosolized pentamidine, or atovaquone
Includes allogeneic stem cell recipients, patients with acute lymphocytic leukemia,
or those receiving alemtuzumab
Febrile neutropenia
Single temperature >38.3 or >38.0 for over 1 hour
Less than 500 neutrophils or <1,000 neutrophils with predicted decline to <500
over next 48-hours
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11.5 Lymphoid Malignancies
Introduction
Solid tumors of the immune system
Lymphocytes transformed to malignant cells. Can be T, B, NK cells. Majority originate from
B-cells
Hodgkins (HL) vs. Non-Hodgkins lymphomas (NHL)
Epidemiology
HL: Bimodal incidence with peaks at 30 years and then after 50 years of age
NHL: More frequent in elderly and in males
Diagnosis
Biopsy (incisional or excisional), immunohistochemistry or flow cytometry
If no accessible nodes, can do FNA or core needle biopsy
If isolated splenomegaly, often recommend splenectomy
Imaging: CT chest, abdomen, and pelvis. Otherwise, PET-CT
Labs: CBC with differential, CMP, LDH, ESR, peripheral smear
Hodgkins Lymphoma (HL)
~ 8,000 new diagnoses each year in the US
Reed Sternberg cell: Malignant B-cells that express CD15 & CD30
4 types of HL: Nodular sclerosis, mixed cellularity, lymphocyte deplete, lymphocyte rich
Nodular sclerosing is most common in the US
Symptoms
Non-tender lymphadenopathycommonly in neck, axilla, supraclavicular area
Systemic B symptoms
Diagnosis
CBC, ESR, bone marrow biopsy, CT chest, abdomen, and pelvis
Treatment
Early Stage HL (Stage I: Single lymph node; Stage II: 2 or more nodal regions on same
side of diaphragm)
Chemotherapy with ABVD (anthracycline, bleomycin, vinblastine, dacarbazine) and
IFRT (involved field radiation therapy)
BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine,
procarbazine, and prednisone)
85 to 95% disease free survival at 5 years
Advanced Stage HL
Chemotherapy with ABVD, and if bulky disease, radiation therapy
Relapsed HL
If relapse >1 year, systemic chemotherapy
If relapse <1 year, aggressive chemotherapy. Consider autologous stem cell
transplantation (auto SCT)
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Diagnosis
CBC, CMP, peripheral blood smear, flow cytometry, bone marrow biopsy, LDH, uric acid,
beta-2 microglobulin, SPEP, CT chest, abdomen, and pelvis
Absolute lymphocytosis > 4 x 10^9/L
CD5 and CD23 antigen positive
Smear may show smudge cells
Treatment
Asymptomatic - watchful waiting
Bone marrow failure with developing cytopenias - various treatments including:
fludarabine, chlorambucil, cyclophosphamide, rituximab
Complications
Often develop autoimmune hemolytic anemia, autoimmune thrombocytopenia (ITP),
red cell aplasia, and/or hypogammaglobulinemia that require treatment per standard
guidelines, irrespective of CLL treatment
Risk Factors
Genetics
BRCA-1 - involved in gene repair. 60 to 80% lifetime chance of breast cancer if mutated
BRCA-2 - increases risk
Li-Fraumeni syndrome - inherited mutations in p53 tumor suppressor gene. Increases
risk of breast cancer as well as other cancers
Protective: Late menarche, earlier 1st pregnancy, earlier menopause. Decreased lifetime
exposure to estrogen
Increased risk: Smoking, moderate alcohol intake, nulliparity, previous radiation therapy in
younger women
Risk Reduction
If strong family history, especially if Ashkenazi Jewish decent, recommend genetic
counseling
If BRCA-1 or BRCA-2 (+), prophylactic mastectomy can significantly reduce risk;
oophorectomy also reduces risk
Screening mammogram with or without clinical breast exam every 1 to 2 years for >40
years of age
Possible benefits: Decreased fat diet, decreased alcohol consumption, weight loss,
exercise
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Metastatic Breast Cancer
If prior lumpectomy, consider mastectomy and radiation
Hormonal therapy
If post-menopausal ER (+) and:
>1 year past previous anti-estrogen therapy aromatase inhibitor or SERM
<1 year past previous anti-estrogen therapy aromatase inhibitors are first-line
pre-menopausal ER (+):
If
LHRH agonist + SERM
Consider oophorectomy
If rapidly progressive or refractory disease: Combination chemotherapy
If Her2/neu (+): Trastuzumab + chemotherapy
If metastases to bone: Include bisphosphonates e.g., zoledronate or pamidronate
Prognosis
Depends on stage of cancer (TNM), grade, patients age, and performance status, and
recurrence of disease
Nottingham Prognostic Index is used after surgery for breast cancer
NPI = [0.2 x S] + N + G
Where: S is the size of the index lesion in centimetres. N is the number of lymph nodes
involved: 0 =1, 1-3 = 2, >3 = 3. G is the grade of tumour: Grade I =1, Grade II =2, Grade III
=3
Score 5-year survival
>/=2.0 to </=2.4 93%
>2.4 to </=3.4 85%
>3.4 to </=5.4 70%
>5.4 50%
Risk Factors
Age
Family history
Cigarette smoking and alcohol consumption
Postmenopausal hormone-replacement therapy
Protective: Increasing parity, oral contraceptive use, oophorectomy
Lactaction, incomplete pregnancies, and surgeries (hysterectomy and tubal ligation) may
confer a week protective effect against the cancer
Infertility may contribute as a risk for nulliparous women
Diagnosis
Family history: Breast, ovarian, and/or colon cancer
Abdominal swelling and/or tenderness
Abnormal vaginal bleeding
Pelvic or transvaginal ultrasounds - mass may be present
CA-125 blood test
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Treatment
Surgery to remove the ovaries, along with additional areas where it has spread (often
spread to omentum)
Chemotherapy may be intitial treatment in advanced cases
Follow-up with pelvic exam and serum CA-125 blood tests
Prevention
HPV vaccine for women 9-26; however, does not protect against ALL strains and does not
treat exisiting HPV
Treatment
Loop electrosurgical excision procedure (LEEP)
Cervical cone biopsy
Simple or radical hysterectomy
Radiation
Chemotherapy
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Subsets of Treatable Cancer of Unknown Primary
Axillary Lymphadenopathy in Women
Women presenting with this should be treated as Stage II or III breast cancer
MRI can help identify primary even when mammogram is normal
Inguinal Lymphadenopathy
Lymph node biopsy of most accessible node
Superficial lymph node dissection with or without subsequent chemotherapy based on
cell type seen in pathology
Cervical Lymphadenopathy
Panendoscopy, panorex, CT head and neck for diagnosis
If squamous cell seen on pathology, treat as head and neck cancer
Peritoneal Carcinomatosis in Women
Peritoneal carcinomatosis with malignant ascites should prompt treatment for ovarian
cancer
Treat as Stage III ovarian cancer with cytoreductive surgery and chemotherapy
Poorly Differentiated Carcinoma
Young males with midline tumor location in mediastinum and peritoneum, elevated
human chorionic gonadotropin and alpha fetoprotein should be treated as testicular
cancer with poor prognosis
Treat with chemotherapy and surgical resection of residual disease
Pulmonary Issues
Bleomycin induced pneumonitis. Higher risk when associated with: Age >70, higher doses,
underlying pulmonary disease, prior mediastinal radiation, renal dysfunction
Radiation pneumonitis: Risk increases with prior chemotherapy, withdrawal of
corticosteroids
Chemotherapy
Related infertility can be managed by storage of sperm/eggs/embryos
Hormonal imbalance from chemotherapy related side effects can cause significant
morbidity
Problems with cognition have been reported after chemotherapy
Radiation therapy to the neck should prompt evaluation for hypothyroidism
Gastric Cancer
Predisposing Factors
Male sex
Age
Smoking
Family history of gastric cancer
Familial adenomatous polyposis
H. pylori infection (main cause)
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Partial gastrectomy
Atrophic gastritis
High salt intake
Signs and Symptoms
Anemia
Melena
Hematemesis
Abdominal pain
Palpable abdominal mass
Nausea
Vomiting
Weight loss
Staging and Diagnosis
Upper endoscopy with biopsy is gold standard
AJCC and TNM staging is used
Treatment
Local disease: Stage 0-1b - surgery with curative intent
For resectable disease: Adjuvant therapy is concurrent chemotherapy with 5-FU,
Leucovorin, and radiation therapy
Neoadjuvant therapy is with epirubicin, cisplatin and 5-FU, followed by postoperative
adjuvant therapy
Adjuvant capecitabine plus oxaliplatin treatment after curative gastrectomy should be
considered as a treatment option for patients with operable gastric cancer
Neoadjuvant, adjuvant and radiation therapy have been shown to improve overall survival
Metastatic disease or recurrent disease: Incurable. Single agents epirubicin, oxaliplatin,
capecitabine, docetaxel or combination regimens. No standard regimen exists
Surveillance
Every 3 to 4 months for 3 years followed by annual visits
CEA, CT scans, bone density, serum B12 levels, iron, folate, calcium level monitoring for
post gastrectomy patients
Esophageal Cancer
Two types : squamous cell, and adenocarcinoma
Risk factors for adenocarcinoma: smoking, obesity, GERD
Risk factors for squamous: smoking, alcohol, unhealthy diet
BOTH: Age> 60, male sex, HPV, Plummer-Vinson Syndrome
Signs and Symptoms
Dysphagia, initially for solids then to liquids, weight loss, odynophagia, retrosternal pain,
hoarseness of voice, cough
Staging and Diagnosis
Barium swallow, esophagogastroduodenoscopy with biopsy is gold standard
AJCC TNM staging
Treatment
Esophagectomy in lymph node negative disease
Preoperative chemoradiation therapy followed by surgery increases overall survival when
compared to surgery alone
Metastatic disease: No standard regimen
Pancreatic Cancer
Subtypes: adenocarcinoma, and neuroendocrine tumors; Fourth cause of cancer death in the U.S
Predisposing Factors
Smoking, family history
Chronic pancreatitis, diabetes, obesity
Signs and Symptoms
Abdominal pain, pain radiating to the back, bloating, nausea
Weight loss, anorexia
Jaundice
Staging and Diagnosis
High-resolution spiral dynamic phase CT abdomen, liver function panel, endoscopic
ultrasound
AJCC TNM staging
Treatment
Whipples procedure for resectable disease (cholecystectomy, partial gastrectomy,
resection if proximal jejunum, portion of pancreas and gastrojejunostomy)
Neoadjuvant chemotherapy for locally advanced resectable, and locally advanced
unresectable disease with gemcitabine and 5-FU
Metastatic disease: Gemcitabine is standard regimen. Addition of Erlotinib has shown
minimal improvement in overall survival
Surveillance
History and physical examination every 3 months for 1st 2 years, every 6 months for
subsequent 3 years
CA 19-9, CT chest/abdomen/pelvis at each follow-up
Prognosis
Poor:
<5% 5-year survival rate
25% 1-year survival rate
My suggested readings
American Joint Committee in Cancer: AJCC: https://cancerstaging.org/Pages/default.
aspx
National Guidelines Clearinghouse.
http://www.guideline.gov/
http://www.Uptodate.com
American Cancer Society : http://www.cancer.gov
Wikipedia, the free encyclopedia, Colorectal cancer
Wikipedia, the free encyclopedia, gastric cancer
Wikipedia, the free encyclopedia, breast cancer
Wikipedia, the free encyclopedia, esophageal cancer
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References & Suggested Readings
1. Rajput, A., Romanus, D., Weiser, M.R., terVeer, A., Niland, J., Wilson, J., Skibber J.M., Wong Y.N.,
Benson A., Earle C.C., Schrag D. (2010) Meeting the 12 lymph node (LN) benchmark in colon cancer.
Journal of Surgical Oncology. 102(1), 3-9.
2. Dotan E, Cohen SJ. (2011). Challenges in the management of stage II colon cancer. Seminars in
Oncology. 38(4), 511-20.
3. Andr T, Boni C, Navarro M, Tabernero J, Hickish T, Topham C, Bonetti A, Clingan P, Bridgewater J,
Rivera F., (2009) Improved overall survival with oxaliplatin, fluorouracil, and leucovorin as adjuvant
treatment in stage II or III colon cancer in the MOSAIC trial. Journal of Clinical Oncology. 27(19),3109-
16.
4. Thrlimann B, Keshaviah A, Coates AS, Mouridsen H, Mauriac L, Forbes JF, Paridaens R, Castiglione-
Gertsch M, Gelber RD, Rabaglio M, Smith I, Wardley A, Price KN, Goldhirsch A. (2005). A compari-
son of letrozole and tamoxifen in postmenopausal women with early breast cancer. New England
Journal of Medicine. 253:2747-57.
5. Su Y, Yang WB, Li S, Ye ZJ, Shi HZ, Zhou Q. (2012). Effect of angiogenesis inhibitor bevacizumab on
survival in patients with cancer: a meta-analysis of the published literature. PLoS One. 7(4):e35629.
6. Cunningham D, Allum WH, Stenning SP, Thompson JN, Van de Velde CJ, Nicolson M, Scarffe
JH, Lofts FJ, Falk SJ, Iveson TJ, Smith DB, Langley RE, Verma M, Weeden S, Chua YJ., (2006)
Perioperative chemotherapy versus surgery alone for resectable gastroesophageal cancer. MAGIC
Trial Participants. New England Journal of Medicine. 355(1),11-20.
7. Slamon DJ, Leyland-Jones B, Shak S, Fuchs H, Paton V, Bajamonde A, Fleming T, Eiermann W,
Wolter J, Pegram M, Baselga J, Norton L. (2001). Use of chemotherapy plus a monoclonal anti-
body against HER2 for metastatic breast cancer that overexpresses HER2. New England Journal of
Medicine. 344(11):783-92.
8. Bang YJ, Kim YW, Yang HK, Chung HC, Park YK, Lee KH, Lee KW, Kim YH, Noh SI, Cho JY, Mok YJ,
Kim YH, Ji J, Yeh TS, Button P, Sirzn F, Noh SH., (2012). Adjuvant capecitabine and oxaliplatin for
gastric cancer after D2 gastrectomy (CLASSIC): a phase 3 open-label, randomized controlled trial.
Lancet. 379(9813), 315-21.
9. Kelsen DP, Ginsberg R, Pajak TF, Sheahan DG, Gunderson L, Mortimer J, Estes N, Haller DG, Ajani J,
Kocha W, Minsky BD, Roth JA. (1998). Chemotherapy followed by surgery compared with surgery
alone for localized esophageal cancer. New England Journal of Medicine. 339(27),1979-84.
10. Tepper J, Krasna MJ, Niedzwiecki D, Hollis D, Reed CE, Goldberg R, Kiel K, Willett C, Sugarbaker
D, Mayer R. (2008) Phase III trial of trimodality therapy with cisplatin, fluorouracil, radiotherapy,
and surgery compared with surgery alone for esophageal cancer: CALGB 9781. Journal of Clinical
Oncology. 26(7):1086-92.
11. Basic & Clinical Pharmacology, (12th ed.). (2011). McGraw-Hill Medical.
12. Moore MJ, Goldstein D, Hamm J, Figer A, Hecht JR, Gallinger S, Au HJ, Murawa P, Walde D, Wolff
RA, Campos D, Lim R, Ding K, Clark G, Voskoglou-Nomikos T, Ptasynski M, Parulekar W. (2007).
Erlotinib plus gemcitabine compared with gemcitabine alone in patients with advanced pancreatic
cancer: a phase III trial of the National Cancer Institute of Canada Clinical Trials Group. Journal of
Clinical Oncology. 25(15):1960-6.
13. Oettle H, Post S, Neuhaus P, Gellert K, Langrehr J, Ridwelski K, Schramm H, Fahlke J, Zuelke
C, Burkart C, Gutberlet K, Kettner E, Schmalenberg H, Weigang-Koehler K, Bechstein WO,
Niedergethmann M, Schmidt-Wolf I, Roll L, Doerken B, Riess H. (2007). Adjuvant chemotherapy with
gemcitabine vs observation in patients undergoing curative-intent resection of pancreatic cancer: a
randomized controlled trial. JAMA. 297(3):267-77.
14. Harrisons Principles of Internal Medicine, (18th ed). (2011). McGraw-Hill Professional.
15. The Washington Manual Hematology and Oncology Subspecialty Consult, (2nd ed.). (2008).
Lippincott, Williams & Wilkins.
MEDICAL ONCOLOGY149
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NOTES
Yashaswini Yeragunta, MD
Edward Kessler, MD
C o n t e n t s
MELLITUS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
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12.1 ADRENAL GLAND
The adrenal cortex produces 3 classes of steroids: Glucocorticoids (i.e., cortisol),
mineralocorticoids (i.e., aldosterone) and androgen (dehydroepiandrosterone)
The diagnostic test of choice for glucocorticoid excess is the dexamethasone suppression
test
The diagnostic test of choice of glucocorticoid deficiency is cosyntropin stimulation test
Mineralocorticoid deficiency is characterized by renin excess
Mineralocorticoid excess is heralded by renin suppression
Cushing Syndrome
Disorder of glucocorticoids excess, which can either be ACTH driven (pituitary tumor or
ectopic ACTH tumor), non-ACTH driven (adrenal adenoma or hyperplasia), or iatrogenic
(use of exogenous steroids, which is most common)
Most commonly seen in women or prepubertal aged men
McCune-Albright syndrome is glucocorticoid excess, non ACTH driven, associated with
polyostotic fibrous dysplasia, unilateral caf-au-lait spots, and precocious puberty
Clinical Features
Common symptoms include: Skin manifestations (bruising, striae, acne), diabetes, truncal
obesity, proximal myopathy, hirsutism, and hypertension
Due to hypercoagulable state, they are at high risk for venous thromboembolism
Increased rate of cardiovascular disease and osteoporosis with vertebral fractures are
noted in patients with Cushings even after effective treatment
Diagnosis
See Figure on Diagnosis of Cushing Syndrome
Check ACTH
Confirmatory testing can be done
by low dose dexamthesone
suppression test
HIGH LOW
Mineralocorticoid Excess
Most common etiology is adrenal hyperplasia
Other causes include adrenocortical carcinoma and congenital adrenal hyperplasia
Liddles syndrome is a rare autosomal dominant mutation in sodium channel with features
of aldosterone excess (hypertension, metabolic alkalosis, hypokalemia), but found to have
low renin and aldosterone levels
Diagnosis
Screening for mineralocorticoid excess in setting of hypokalemic hypertension, early
onset of hypertension age prior to 40 years of age and hypertension associated with
drug resistance
Test for renin and aldosterone levels after stoppage for any mineralocorticoid active
drugs (i.e., spironolactone and ACEI/ARB) for at least 4 weeks
The test is positive if ratio is >750 pmol/L: ng/mL per hour with high/normal aldosterone
Aldosterone to plasma renin activity levels >20 in patients off ACEI/ARBS and >10 in
patients on ACEI and ARBS is indicative of hyperaldosteronism
Adrenal Incidentaloma
Defined as an adrenal mass, generally 1 cm or more in diameter that is discovered
serendipitously during a radiologic examination performed for indications other than an
evaluation for adrenal disease
Incidence increases with age, for example incidence between ages 20 to 29 is
approximately 0.2%, as compared with approximately 7% in a patient over 70 years of age
Functional assessment may reveal: Subclinical Cushing syndrome, clinically silent
pheochromocytoma, primary aldosteronism
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Assessment of malignant potential
Size of the mass and imaging are major predictors of malignant potential
Size greater than 4 cm or growth greater than 1 cm on repeat imaging (at 6, 12, 24
month intervals) is commonly recommended to be resected
Adrenal tumors should be removed if they are functional
Imaging phenotype
Lipid content and time of washout aid distinguishing adenomas from non adenomas
Metastatic disease
Carcinomas of lung, breast, colon, esophagus, pancreas, and liver frequently
metastasize to the adrenals and are frequently bilateral
Because of the high cost, FDG-PET is not recommended routinely for evaluation with
no history of malignancy
Fine needle aspiration biopsy
To be used with caution for pheochromocytoma as it can precipitate crisis, hence
definitive biochemical functional assessments should be performed prior to biopsy
Hypopituitarism
Micro are defined as tumors >10 mm radiologically and micro are <10 mm
Pituitary adenomas, radiation, trauma, and surgery are common etiologies of
hypopituitarism
Clinical effects of hypopituitarism depend on concomitant hormone deficiency
Growth Hormone Deficiency
Regulated by growth hormone-releasing hormone
Short stature in children; in adults causes decreased strength, libido, increased body fat,
decreased cardiac output
Treatment with GH replacement improves all the above complaints but can have
unfavorable effects such as arthralgias and fluid retention, hypertension, gynecomastia,
and acromegaly
Diabetes Insipidus
Characterized by deficiency of vasopressin
Suspected in situations with high urine output (greater than 50mL/kg) and urine osmolality
<300 mOsmol/L
Etiology
Central - genetic, inherited, tumor, craniopharyngioma, cancer, infectious causes,
aneurysms, Sheehan syndrome, autoimmune
Nephrogenic - resistance to vasopressin
Drug induced - lithium, demeclocycline, amphotericin B, and antibiotics (i.e.,
aminoglycosides)
Clinical Features
Symptoms include polyuria, polydipsia, enuresis, and excessive thirst
Serum osmolarity <300 mOsmol/L
Water deprivation test:
Measure osmolarity (serum and urine) at baseline and every 2 hours until serum
osmolarity is >295 mOsmol/kg and the patient has lost no more than 3% body
weight. Subsequently, after administration of 5 ug of desmopressin intranasally or
intravenously, the osmolarity is re-measured
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Healthy patients, during water deprivation, will increase urine osmolarity >800 mOsmol/
kg as an appropriate response, whereas patients with DI, will not have a rise in urine
osmolarity >300 mOsmol/kg
In patients with central DI, there will be rise in osmolarity of 50% or greater in osmolarity
in response to desmopressin
In nephrogenic DI, there will be little or no response in urine osmolarity to desmopressin
Treatment
Nephrogenic DI treatment includes high dose desmopressin, thiazides and NSAIDs
(i.e., indomethacin)
Central DI requires desmopressin replacementavailable in subcutaneous, intranasally, or
oral forms
Acromegaly
Caused by excessive GH secretion from pituitary or hypothalamic cause
Clinical Features
Enlargement of nose, lip, tongue, frontal bossing, enlargement of hands and feet,
amenorrhea, cardiomegaly, cardmyopathy, hypertension, diabetes
Diagnosis
Diagnosis is established by IGF-1 levels which has high sensitivity
Direct measurement of GH secretion is futile due to pulsatile nature of GH
Oral glucose tolerance test is confirmatory
Treatment
Surgical resection (transphenoidal approach) is primary treatment for GH secreting
adenomas
Current medical therapies include dopamine agonists (i.e., cabergoline), octreotide
(decreases GH and IGF levels) and pegvisomant (synthetic GH analogue, blocks GH
receptor)
Radiation therapy can be offered in recalcitrant or unresectable cases
Early screening colonoscopy with close follow up every 3 to 5 years is recommended due
high propensity of colonic polyps
Hypothalamus
TRH
Pituitary
T3 + T4
TSH
Liver
T3 + T4
Thyroid
Hypothyroidism
Clinical Features
Depression
Fatigue
Delayed DTR
Bradycardia
Diastolic hypertension
Constipation
Menorrhagia
Dry skin
Thin hair
Cold intolerance
Weight gain
Etiology
Most common cause is iodine deficiency, autoimmune disorders (Hashimotos) and
Iatrogenic
Other causes include medications like iodine, amiodarone, lithium, congenital
hypothyroidism, and dyshormonogenesis, infiltrative disorders (i.e., amyloid, sarcoid and
scleroderma), postpartum thyroiditis
Secondary causes include hypopituitarism or hypothalamic disease (i.e., tumors, surgery,
irradiation, trauma and ischemia)
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Diagnosis
Figure 12.3. Approach to Diagnosis of Hypothyroidism
TSH, T4
MRI brain
Etiologies of falsely low T3/T4: Lab error, cirrhosis, nephrotic syndrome, severe illness,
and medications
Etiologies of falsely high TSH: Exercise, sleep deprivation, recovery from nonthyroidal
illness, elderly
Treatment
Treatment for hypothyroidism is based on levothyroxine replacement
Adults require around 1.7 microg/kg of body weight
The American Thyroid Society recommends starting full replacement in patients younger
than 50 years
Patients 50 years or older should be started at 25-50 mcg daily
Re-evaluate thyroid function studies at 6 to 8 week intervals and titrate levothyroxine
Special situations
Sick euthyroid syndrome noted in the setting of severe stress (shock, surgery,
malnutrition) due to increased metabolism or inhibition of thyroid hormone binding.
Laboratory findings include normal TSH, low T4, and increased reverse T3
Treatment with thyroid hormone replacement is not indicated. Treat the underlying
cause
Thyrotoxicosis
Defined as an excess of thyroid hormones. Thyrotoxicosis is not synonymous with
hyperthyroidism, which is the result of excess thyroid function
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Diagnosis
Figure 12.4. Approach to Diagnosis of Thyrotoxicosis
Check TSH/T4
Low TSH, High FT4 Low TSH, FT4 WHL High TSH, High FT4
Diffuse goiter,
t3 thyrotoxicosis High Normal
dermatopathy,
ophthalmopathy
Subclinical
hypterthyroidism
Radioactive iodine
Graves' disease
uptake scan
Treatment
Graves'
1. Antithyroid medications (methimazole, carbimazole, and PTU)
2. b-blocker for symptom control
3. Therapeutic radioactive iodine
Antithyroid medications (ATM)
All medications inhibit TPO function
Propylthiouracil additionally inhibits deiodination of T4 to T3
Clinical exam, and unbound free T4 every 4 weeks to aid titration of
medications
Thyroiditis
Acute
Rare presentation typically due to infectious etiologiesmost commonly related to
presence of piriform sinus in youth, but also concerning for malignancy in elderly
Typical signs/symptoms: Neck/thyroid pain with radiation to ears
Lab evaluation: TSH/T4 normal; diagnosis guided by biopsy, stain, and culture results
Subacute (De Quervains, Granulomatous, or Viral Thyroiditis)
Viral etiology (mumps, Coxsackie, adenovirus, influenza, echovirus, etc.)
Diagnosis: Clinical presentation vague and similar to URI/pharyngitis
Lab evaluation: Vary by stages, early stage (thyrotoxic phase with elevated T4 and T3,
low TSH; hypothyroid phase with elevated TSH and low T4/T3 and recovery phase with
normal thyroid function tests)
Treatment for pain and fever: Acetaminophen may reduce pain and fever; aspirin or
NSAIDs may treat inflammation and fever; refractory treatment include steroids
Silent
Minimal symptoms hence silent
Most commonly occurs post partum (3 to 6 months); Similar course as subacute
thyroiditis
Initial toxicosis lasts 2 to 4 weeks and hypothyroidism lasts 4 to 12 weeks
Associated with type 1 DM
Painless goiter, differentiated from subacute thyroiditis with normal ESR and +TPO
antibodies
No treatment until symptom of thyrotoxicosis manage with b-blocker; levothyroxine
replacement in hypothyroid phase
Drug Induced
Common inciting agents include interferon alpha, IL-2, and amiodarone
Chronic
Hashimotos thyroiditis: Most commonly seen in women. Usually painful. Autoimmune in
origin
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Riedels thyroiditis: Usually painless goiter with compressive symptoms on the esophagus,
trachea. Hard on palpation and easily mistaken for malignancy
FNA biopsy is usually inadequate and open biopsy is required for diagnosis
Treatment is usually surgical
Sick euthyroid syndrome
Noted mostly in people with critical illness
Impaired conversion of T4 to T3 resulting in normal TSH, normal T4, but low T3 with
elevation in reverse T3. Fluctuations noted in TSH. Deficiencies usually correct after
treating critical illness
Hypophosphatemia
Etiologies: Reduced or impaired absorption in the intestine due to intake of sevelamer or
aluminum containing antacids
Depressed renal reabsorption in primary or secondary hyperparathyroidism and
hypercalcemia of malignancy
Rapid redistribution responsible for low serum availability due to insulin therapy,
catecholamines, intravenous glucose, after parathyroidectomy, leukemic blast crisis, and
catecholamine surge
Clinical manifestations include but are not limited to lethargy, confusion, hallucinations,
paresthesia, including visceral malfunction such as cardiomyopathy and respiratory
compromise due to muscle fatigue
Serious consequences like paralysis and seizures are noted at very low levels (less than
1 mg/dl)
Treatment includes aggressive replacement using sodium or potassium phosphate salts
Treat underlying cause and concomitant electrolyte and nutritional deficits including vitamin D
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Hyperphosphatemia
Most common etiology is renal failureacute or chronic
Also noted in hypoparathyroidism, hypomagnesemia, pseudohypoparathyroidism, crush
injuries, rhabdomyolysis, hyperthermia, and acidosis causing transcellular shifts
Clinical manifestations are due to calcium binding resulting in hypocalcemia, which may
lead to tetany, seizures, etc.
Treatment
Phosphate binders (i.e., sevelamer) limit intestinal absorption
Fluid resuscitation aid renal clearance
Consider hemodialysis in severe and/or symptomatic hypocalcemia
Parathyroid Hormone
Secreted by the parathyroids
Acts on the kidney to produce 1,25 vitamin D, which enhances renal calcium reabsorption
and on the bone osteoclasts to increase serum calcium concentration
Indirectly influences intestinal calcium absorption via 1,25 vitamin D
The serum ionized calcium level closely regulates PTH level through a negative feedback
mechanism
Calcitonin is a PTH antagonist secreted by the thyroid and inhibits osteoclast bone
resorption
12.5 Hypercalcemia
Causes
Hyperparathyroidism mainly causing raised PTH level, resulting in raised calcium levels
Granulomatous diseases like sarcoidosis and lymphomaelevated vitamin D levels leads to
increased absorption
Genetic causes such as familial hypocalciuric hypercalcemia with impaired parathyroid and
renal calcium receptors
Increased intake (i.e., milk-alkali syndrome)
Malignancy (i.e., breast cancer, myeloma, lytic lesions)
Medications (i.e., thiazide diuretics)
Clinical Manifestations
Rapid increase in calcium level has more pronounced clinical presentations, including
lethargy, altered mental status, seizures, and may result in coma if untreated
Chronic elevation of calcium level can lead to varied presentation like renal stones, gastritis,
pancreatitis, constipation, and fractures
Laboratory Detection
Intact PTH assay helpful to diagnose various etiologies such as primary hyperparathyroidism
or rapid bone turnover
Treatment
IVF resuscitation
Loop diuretics (i.e., furosemide) should be used only after adequate volume replacement
12.6 HYPOCALCEMIA
Serum calcium level drops by 0.8 mg/dl for every 1 gm/dl drop in serum albumin
It is important to distinguish true hypocalcemia from falsely low secondary to low albumin
Most common etiology is chronic kidney disease, with its low vitamin D level and
hyperphosphatemia
Also noted in electrolyte abnormalities like hypomagnesemia, hypoparathyroidism from
genetic causes, and chronic vitamin D deficiency from poor PO intake
Clinical Manifestations
Includes tetany, carpopedal spasm, Chvosteks sign (tapping of facial nerve leading to
twitching)
Prolong QT leading to life-threatening ventricular abnormalities
Laboratory evaluation begins with measuring serum albumin and Ionized calcium level to
assess accurately hypocalcemia
Next is to measure PTH level and determine if there is an appropriate response to
hypocalcemia
Vitamin D level should be assessed as well as other possibilities including CKD
Treatment
Calcium carbonate supplements offer the highest elemental calcium
Per FDA, dietary calcium requirements around 1,000 mg for men and premenopausal
women
Achlorhydric patients (like those on PPI) have difficulty absorbing calcium
In case of dangerously low Ionized calcium (ionized calcium below 1.15 mg/dl) with EKG
abnormalities, monitor patients with aggressive replacement using intravenous infusion of
calcium gluconate/chloride with dextrose
Concomitant vitamin D supplementation is beneficial
Less expensive ergocalciferol or the more expensive active form of vitamin D3 can be
utilized
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12.7 DIAGNOSIS AND CLASSIFICATION OF DIABETES MELLITUS
Diabetes mellitus is a disease based on insulin deficiency or increased resistance to insulin
actions. It is presently classified on the basis of pathogenesis.
Type 1: Immune medicated, associated with beta cell destruction
Type 2: Varying degrees of insulin resistance with subsequent deficiencies secondary to
secretory defect
Gestational diabetes mellitus: Onset of hyperglycemia during gestation
Other types: Genetic defects leading to maturity onset diabetes of the young
Diseases of exocrine pancreas secondary to chronic pancreatitis, cystic fibrosis, and
hemochromatosis
Diagnosis
Present if any 1 of the following are there:
New onset Hba1c >/=6.5 gm% or fasting blood glucose >/=126 mg/dl or random glucose
>/=200 mg/dl
2 hour plasma glucose is greater than/equal to 200 mg/dl
For gestational diabetes:
Fasting > equal to 92 mg/dl
1 hr > equal to 180 mg/dl
2 hr > equal to 153 mg/dl
Performed at 24 weeks of gestation
Treatment
Goals
Hemoglobin A1C: <7.0 gm%
Pre-prandial plasma glucose: 70-130 mg/dl
Postprandial plasma glucose: <180 mg/dl
Blood pressure: <130/80 mmHg
LDL: 100 mg/dl (with extensive cardiovascular disease <70 mg/dl)
HDL-cholesterol
Men: >40 mg/dl
Women: >50 mg/dl
Triglycerides: <150 mg/dl
Oral Hypoglycemic Agents
Insulin
Short acting - regular insulin
Onset of action: 30 min
Duration of action: 4 to 8 hrs
Lispro insulin
Onset of action: <15 min
Peak of action: 1 hr
Duration of action: 3 to 5 hrs
Intermediate acting - NPH insulin
Onset of action: 2 to 4 hrs
Peak of action: Within 8 to 10 hrs
Duration of action: 10 to 18 hrs
Complications
Acute
DKA (Diabetic Ketoacidosis)
Secondary to insulin deficiency from various causesmost commonly dietary, medication
non-compliance, infection, pancreatitis, and other stress states. Imbalance between
endogenous production and glucagon overproduction leading to hyperglycemia, ketosis,
and anion gap metabolic acidosis
Laboratory findings: Blood glucose >250 mg/dl, serum ketones at a dilution of 1:8 or
greater, hyperkalemia (serum potassium of 5-8 meq/slight hyponatremia, serum sodium
of approximately 130 meq/l), hyperphosphatemia (serum phosphate level of 6-7 mg/dl).
Acidosis maybe severe with pH 6.9-7.2 and serum bicarbonate between 5-15 meq/dl
HONK (Hyperosmolar Nonketotic State)
Secondary to similar etiologies as DKA. Relative insulin deficiency but without significant
ketone production
Laboratory findings: Hyperglycemia >600 mg/dl, serum osmolarity >310 mOsm/kg,
bicarbonate >15 meq/L and normal anion gap
Treatment: Similar for both conditions essentially based on degree of severity with goals
to restore plasma volume deficit, reduce blood glucose, and replenish electrolytes
Plasma Volume Deficit
Around 4-5 L. Usually with 0.9% normal saline or 0.45% normal saline (if serum sodium is
>150 mEq/L). Switch to dextrose 5% once blood glucose <250 mg/dl
Insulin Replacement
IV regular insulin is preferred. Continuous drip is to be titrated based on hourly blood
sugar levels. In HONK, relatively less overall requirement in comparison to DKA
Electrolyte replacement, especially potassium and phosphorus, as needed
Chronic
Ophthalmological complications: Retinopathy, macular edema, cataracts
Nephropathy: Proteinuria/microalbuminuria 30 to 300 ug albumin/24 hrs to overt
proteinuria >300 mg/d. ACEI and ARB. This can be effectively type 4 renal tubular
acidosis, ESRD
Neuropathy
Distal symmetric nephropathy
Isolated peripheral neuropathy
Painful diabetic neuropathy
Autonomic neuropathy
Cardiovascular disease: Coronary artery disease
Gastrointestinal: Gastroparesis
Sexual dysfunction
Dermatologic manifestations: Acanthosis nigricans
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12.8 REPRODUCTION
Male Physiology
Hypothalamus produces gonadotropin releasing hormone (GNRH) which acts on the
pituitary to produce luteinizing hormone (LH) and follicular stimulating hormone (FSH)
LH stimulates Leydig cells to produce testosterone
FSH stimulates Sertoli cells which aid in spermatogenesis and inhibin B, which provides
feedback inhibition on FSH release
Most testosterone bound to sex hormone binding globulin (SHBG); less than 5% of
testosterone remains unbound or bound to albumin
Hypogonadism
Etiologies
Primary etiologies: Genetic (i.e., Klinefelters), androgen resistance, infection, medication/
toxins (alcohol, azoles, androgen-inhibition, chemotherapy, etc.), and trauma
Secondary etiologies: Multifactorial and affecting hypothalamus/pituitary including
masses (tumors), systemic/infiltrative disease (hemochromatosis, amyloid, etc.), ischemic
insults, malnutrition, and medication/toxins
Clinical Presentations
In pre-/peri-pubertal states, features include small testes, delayed/arrested maturation,
infertility, lack of secondary sexual characteristics
In post pubertal states: Infertility, erectile dysfunction, decreased libido, small testes,
fatigue, loss of secondary sexual characteristics, and muscular atrophy
Laboratory Evaluations
GNRH, LH, FSH, prolactin, free testosterone levels; may consider karyotype if elevated
GNRH
Treatment
Address primary etiology; testosterone replacement
Signs/symptoms of
hypogonadism
Free testosterone
Normal or elevated
Low testosterone
testosterone
Suspect androgen
Obtain FSH, LH
resistance
Elevated FSH
Low FSH and LH Normal FSH, LH
and/or LH
Testosterone Replacement
Preparations include intramuscular injections and topical applications
Requires serial assessment of testosterone levels, hemoglobin, hematocrit, lipids, and PSA
Contraindications: Breast cancer, hypersensitivity, and prostate cancer
Erectile Dysfunction
Etiologies
Organic: Inflammation/infection, vaso-occlusive disease, trauma, endocrine disorders (DM,
hypogonadism, hyperprolactinemia), drug/medications
Psychogenic: Anxiety, depression, stress-related disorders
Clinical Presentations
Insufficient erection for intercourse; often times associated with older age, systemic
disease (especially atherosclerosis, peripheral arterial disease, DM), and smoking history
Diagnosis
History/exam (secondary sexual characteristics, hair distribution, heralding signs of other
systemic illnesses, cardiovascular assessment, etc.)
Labs: Testosterone level, prolactin level, GNRH level, BMP, CBC, and lipids
Other tests: Nocturnal tumescence, vascular studies (Doppler, angiography), neurological
evaluation, and psychological evaluation
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Treatment
Address underlying etiology
Patient education: Lifestyle counseling, avoid recreational drugs/substances
Therapies
Oral phosphodiesterase inhibitors (sildenafil, vardenafil, etc.)
Onset within 60 to 120 min
Caution with patients with renal dysfunction, co-administration with medications
(nitrates, alpha blockade, azole antibiotics, and other medications metabolized by
CYP3A4 pathway)
Side effects: Bluish visual disturbance and headache
Other therapies: Intraurethral suppositories, intracavernosal injections, prosthesis
implantation, vacuum assist devices
Female Physiology
LH acts on theca cells which release androgens (i.e., androstenedione, testosterone)
FSH acts on granulosa cells leading to increased aromatase and inhibin B; aromatase
converts androgens to estradiol leading to ovulation
Granulosa cells form corpus luteum post ovulation, which secrete progesterone
Unless the egg is fertilized, the corpus luteum regresses, leading to progesterone
withdrawal and then menstruation
Polycystic Ovarian Syndrome (PCOS)
Commonly diagnosed in puberty
Etiologies
Unclear, related to androgen hormone imbalance, amenorrhea, obesity, insulin resistance,
and hirsutism
Clinical Presentation
Irregular menstrual cycles, virilization (i.e., voice changes, decreased breast size, acne, etc.)
Diagnosis
Rotterdam criteria - 2 of 3 of the following:
Irregular/absent ovulation
Elevated androgen levels
Enlarged ovaries with at least 12 follicles
R/O Cushings, hyperprolactinemia, and CAH (congenital adrenal hyperplasia)
Additional tests: Glucose oral tolerance test, fasting lipids
Treatment
Planning for fertility: Metformin (off-label); clomiphene second line
No desire for pregnancy: First-line is spironolactone and oral contraceptive (progesterone
only); second-line: Androgen antagonist
Improvement in hirsutism: Requires at least 3 months of therapy and mechanical removal
(waxing, shaving, etc.)
Amenorrhea
Primary: No menses by age 16 years
Secondary: No menses for 3 months in a female with previous menstrual cycles
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References & Suggested READINGS
1. Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007;
356(6):601-610. [PMID: 17287480]
2. Young WF Jr. Management approaches to adrenal incidentalomas: a view from Rochester,
Minnesota. Endocrinol Metab Clin North Am. 2000;29:159-185.
3. Grumbach MM, Biller BM, Braunstein GD, et al. Management of the clinically inapparent adrenal mass
(incidentaloma). Ann Intern Med. 2003;138:424-429.
4. David M. Nathan, MD, John B. Buse, MD, PHD, Mayer B. Davidson, MD, Ele Ferrannini, MD,
Rury R. Holman, FRCP, Robert Sherwin, MD and Bernard Zinman, MD. Medical Management of
Hyperglycemia in Type 2 Diabetes: A Consensus Algorithm for the Initiation and Adjustment
of Therapy. A consensus statement of the American Diabetes Association and the European
Association for the Study of Diabetes.
5. Jameson JL. Chapter 338. Principles of Endocrinology. In: Longo DL, Fauci AS, Kasper DL,
Hauser SL, Jameson JL, Loscalzo J, eds. Harrisons Principles of Internal Medicine. 18th ed. New
York: McGraw-Hill; 2012. http://www.accessmedicine.com/content.aspx?aID=9139719. Accessed
September 12, 2012.
6. McPhee S, Papadakis M., Rabow M. Current Medical Diagnosis and Treatment. 5th ed. 2012
7. Revised American Thyroid Association Management Guidelines for Patients with Thyroid Nodules
and Differentiated Thyroid Cancer, November 2009.
8. Bahn R., Burch, H., Cooper D., Garber J., Greenlee C., Klein I., Laurberg P., McDougall R., Montori
V., Rivkees S., Ross D., Sosa J., Stan M. Hyperthyroidism and Other Causes of Thyrotoxicosis:
Management Guidelines of the American Thyroid Association and American Association of
Clinical Endocrinologists. The American Thyroid Association and American Association of Clinical
Endocrinologists. Taskforce on Hyperthyroidism and Other Causes of Thyrotoxicosis. Thyroid, V. 21,
Num. 6, 2011.
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NOTES
Saakshi Khattri, MD
C o n t e n t s
13.2 MEDICATIONS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
13.4 Osteoarthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
13.5 Fibromyalgia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
RHEUMATOLOGY/ORTHOPEDICS1
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13.1 Patient with Suspected Rheumatic Disease
Localized vs. Diffuse Rheumatic Pain
Localized rheumatic pain
Arising from joint or periarticular structures (bursitis, tendinitis, or muscle involvement
for e.g., rotator cuff tear)
Diffuse rheumatic pain
Seen in fibromyalgia
Pain in multiple joints (e.g., RA, Psoriatic arthritis)
Approach to Patient
Duration of symptoms: Acute or chronic
Number of joints involved: Single (monoarticular), 2 to 4 (oligoarticular), or >5 (polyarticular)
Symmetric vs. asymmetric
Constitutional symptoms: Fevers, chills, malaise, weight loss, and myalgias
Extra-articular involvement: Rash is common in several rheumatic conditions, e.g., malar
rash in SLE, purpura in some vasculitis
Inflammatory vs. noninflammatory (elevated ESR, CRP)
Comorbidities
Laboratory Evaluation
Routine blood work like CBC, comprehensive metabolic panel (creatinine, liver functions,
albumin)
Thrombocytosis, hypoalbuminemia, anemia may suggest an systemic inflammatory process
Leukopenia especially lymphopenia is seen in SLE
Inflammatory markers (ESR, CRP)
Based on history and exam, testing for antibodies like ANA, SSA, SSB, anti-Smith, anti-
dsDNA, ANCA, RNP, anti-Jo1 is done when one suspects a rheumatic condition (discussed
in detail in later sections)
Synovial fluid analysis: WBC count, crystals, gram stain, and culture if septic arthritis is a
concern
Urine analysis looking for protein and RBC casts (in patients with SLE, suspected vasculitis)
Radiological Evaluation
X-ray studies of involved joints (looking for periarticular osteopenia, erosions, soft tissue
swelling, osteophytes)
Musculoskeletal ultrasound is increasingly being used to image joints and soft tissues and
perform ultrasound guided joint aspirations and steroid injections
MRI can be used in certain conditions, e.g., diagnosis avascular necrosis of a joint or
sometimes in RA
RHEUMATOLOGY/ORTHOPEDICS3
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4. Psoriatic arthritis
5. Sarcoidosis
6. SLE
7. Vasculitis like Kawasaki, Cogan syndrome
8. Inflammatory bowel disease
Scleritis
1. Rheumatoid arthritis
2. Wegeners vasculitis
3. Inflammatory bowel disease
4. Ankylosing spondylitis
5. Relapsing polychondritis
6. Behets disease
13.2 Medications
Acetaminophen
Inhibits prostaglandin synthesis centrally, resulting in analgesic effects and alleviation of pain
Acetaminophen is well tolerated when used as directed
May cause International Normalized Ratio (INR) elevation when given concurrently with
warfarin
When communicating with patients about the use of OTC pain medications and discussing
responsible dosing, it is important for you to:
Know the precise amounts of all substances in an OTC formulation
Know the maximum daily dose of all agents contained in OTC formulations
Monitor renal and liver function periodically
Stay abreast of the latest evidence and dosing in order to give patient up-to-date
advice and recommendations
Counsel patients about the reason for use of the medication, frequency of
administration, anticipated effect on symptoms, and possible drug interactions and
potential adverse effects
Counsel patients to follow the labeled dosage and to not take more than the
recommended labeled dose or the dose that is recommended by you, as this will help
patients to prevent reaching toxic dose levels
Advise patients to communicate the quantity of OTC medications used
Emphasize the importance of reading the package labeling sections labeled Warnings
and Directions. One of the FDA proposed changes requires that all OTC products
containing NSAIDs or acetaminophen (including combination products) to list these
ingredients on the products principal display panel and to also specify the potential
for liver toxicity (in the case of acetaminophen) or GI complications (in the case of
NSAIDs) when these products are taken with 3 or more alcoholic drinks daily
Educate parents and caregivers to keep OTC pain medications out of the reach of
young children by storing these medicines in a high location that is out of the childs
eye sight
Instruct patients to avoid using more than 1 medicine that contains the same active
ingredient
Anti-inflammatory Agents
NSAIDs: Inhibit cyclooxygenase (COX) thereby decreasing prostaglandins with resultant
anti-inflammatory effects
GI side effects (gastritis, dyspepsia, peptic ulcer disease) are the most common
complications
Need to monitor electrolytes and renal functions
Selective COX-2 inhibitors are associated with adverse cardiovascular outcome
Corticosteroids: Anti-inflammatory effects through various mechanisms (upregulation of
the production of anti-inflammatory proteins, inhibit pro-inflammatory cytokines, decrease
eosinophils, and migration of neutrophils to sites of inflammation)
Intravenous pulse methylprednisolone used for severe life threatening complications of
SLE, vasculitis, and myositis
High dose prednisone 1 mg/kg/d used for controlling severe manifestations of
rheumatic conditions
Prolonged use of steroids results in suppression of adrenal glands
Side effects include GI complications, thinning of skin, osteoporosis, and increased risk
of infections
RHEUMATOLOGY/ORTHOPEDICS5
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Leflunomide: Inhibits dihydro-orotate dehydrogenase
Daily dosing 10-20 mg
Has a long half-life
If women on leflunomide decide to get pregnant then there is a need to administer
cholestyramine to clear leflunomide from system
Azathioprine: Blocks purine synthesis
Nausea and vomiting are common side effects
Enzyme thiopurine-S-methyltransferase (TPMT) deactivates active metabolite of
azathioprine and should be measured prior to starting therapy, deficiency of that
enzyme is an absolute contraindication to starting therapy
Bone marrow suppression is a rare complication
Used to treat SLE, rheumatoid arthritis, as steroid sparing agent in myositis, can be
used in maintenance phase of lupus nephritis and vasculitis
Dose is 50-150 mg/d
Cyclophosphamide: Depletes B- and T-cells
Used to treat systemic vasculitis, lupus nephritis, ILD occurring as a complication of
systemic sclerosis
Can be given as daily oral dosing or monthly infusions (NIH protocol) or infusions every
2 weeks (EURO-lupus protocol)
Oral dose is 2 mg/kg/d
Intravenous dose is 500-1,000 mg/m2 body surface area
Dose should be adjusted based on creatinine clearance
Fall in WBC is noted 7 to 10 days after IV administration and if WBC level falls below
1,500 then dose should be reduced on subsequent infusions
Ovarian failure is the most serious side effect of this therapy and risk increases with age
of women
Mycophenolate mofetil: Inhibits inosine monophosphate dehydrogenase
Used to treat SLE, myositis, and vasculitis
1.5-3 g/d is standard dosing
GI side effects are common
Teratogenic to fetus
Cyclosporine: Inhibits T-cells
Used to treat RA and SLE
Elevations in BP and worsening renal functions are some of its side effects
Daily dose is 2.5 mg/kg-4.5 mg/kg
Biologic Agents
Tumors necrosis factor a inhibitors: Include etanercept, adalimumab, infliximab, golimumab,
certolizumab
Given as weekly or monthly subcutaneous injections (infliximab is given as a monthly
infusion)
Used to treat RA, psoriatic arthritis, ankylosing spondylitis
Associated with reactivation of TB, so checking for latent TB is important before
starting therapy
In rare cases, use is associated with a demyelinating syndrome involving CNS
Risk of cancer such as lymphoma has been noted with use
Abatacept: Inhibits T-cell costimulation by binding to B7 protein
Disease Course
Starts in small joints of the hands progressively involving larger joints
Early in the disease soft tissue synovial swelling is seen in the joints
If left untreated, later in the disease erosions develop
In late established disease typical RA deformities arise (ulnar deviation of fingers, swan-neck
deformity, boutonnieres deformity, Z-shaped thumb)
Epidemiology
1% of the population
Females:Males = 3:1
Women in child-bearing age group, men in 6th to 8th decade
Increases with age
Prognostic Factors
Poor prognostic markers are:
RF positivity
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Poor functional status
Extra-articular involvement
Erosive disease
Persistently elevated ESR/CRP
Diagnosis
Clinical Manifestations
Symmetric joint inflammation
Most commonly there is small joint involvement (wrists, MCPs, PIPs)
Typical deformities like ulnar deviation, swan-neck, boutonnieres deformity, Z-shaped
thumb
Morning stiffness lasting >60 min
Involvement of large joints occurs later in the disease - Bakers cyst can be seen in the
knees posteriorly
Evaluation
Routine labs like CBC (looking for anemia, thrombocytosis), liver function test could show
a globulin gap
ESR, CRP: Inflammatory markers
Specific antibodies: Rheumatoid factor, anti-CCP antibody
Radiology: X-rays of affected joints could show erosions, periarticular osteopenia
Management
NSAIDs: Symptomatic relief, minor anti-inflammatory role
Corticosteroids: Most potent anti-inflammatory agents in RA, fast acting
Most commonly used steroid is prednisone
Use lowest dose possible for smallest duration of time to control inflammation and
prevent side effects
May need GI prophylaxis to prevent gastritis with steroid use (PPI, H2 blockers)
Need prophylaxis for osteoporosis if >7.5 mg being used daily for >3 months
13.4 Osteoarthritis
Pathophysiology
Damaged cartilage is unable to repair itself as a result; there is progressive cartilage
degradation with time
There is sclerosis of the underlying bone
Growth of osteophytes at margins
Joint space narrowing due to cartilage loss
Risk Factors
Increasing age
Previous joint injury
Obesity
Sex: Woman have greater risk of OA compared to men
African Americans have greater risk of OA compared to Caucasians
Family history
Clinical Features
Pain in affected joint that increases with activity, improves with rest or OTC analgesics
Stiffness in affected joint that can be seen in the morning or after periods of inactivity but
lasting less than 30 min
Joint instability especially a feeling of legs giving way in knee OA
In knee OA complaints of pain upon walking up or down the stairs
In hand OA with disease progression holding, gripping, opening jars become increasingly
difficult activities to do
Crepitus over affected joint
Bony enlargement of affected joint
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Classification
Primary osteoarthritis: Results from increasing age, family history
Secondary osteoarthritis: Pre-existing conditions that cause cartilage damage like metabolic
disorders (hemochromatosis, Wilsons disease, alkaptonuria), or connective tissue disorders
like Marfan or Ehlers-Danlo syndromes
Erosive inflammatory osteoarthritis: A type of OA where erosions and increased
inflammatory markers are seen
Diagnosis
Examination of affected joint may reveal crepitus
Imaging studies reveal joint space narrowing, osteophytes, bony sclerosis, subchondral cysts
Negative RF, CCP
Synovial fluid aspiration reveals non-inflammatory fluid with WBCs <1,000
13.5 Fibromyalgia
Introduction
Wide spread pain in all 4 quadrants for longer than 3 months
Insufficient, poor, or non restorative sleep
Fatigue
Personality changes
Epidemiology
Prevalence is 3 to 5% amongst women, 1% amongst men
Treatment
Nonpharmacologic therapy: Aquatic therapy, aerobic exercises, tai-chi, yoga, meditation,
biofeedback therapy
Classification
Ankylosing Spondylitis: Inflammatory low back pain associated with morning stiffness >30
min that improves with exercise and recurs with rest
Schobers test <5 cm increase on flexion at LS spine
Ankylosis at cervical spine results in increased occipito-wall distance
Impaired chest expansion <5 cm due to involvement of costovertebral and
costochondral joints
Inflammation at tendon insertions resulting in enthesitis (ischial tuberosity, iliac crests,
greater trochanter, Achilles tendon)
Ocular involvement in form of uveitismost common extra-articular finding
Radiological findings: Sacroiliitis and subsequent fusion of SI joints, shiny corners or
Romanus lesions as a result of inflammation at the site of insertion of annulus fibrosis
on vertebral bodies, squaring of vertebrae, syndesmophytes, and finally bamboo spine
formation
Reactive arthritis: Triggered by bacterial infection in the genitourinary or gastrointestinal
tract
Develops 2 to 4 weeks after gastroenteritis from Salmonella, Shigella, Yersinia,
Clostridium difficile, or Campylobacter or genitourinary infection from chlamydia
Triad of conjunctivitis, urethritis, and arthritis
Dactylitis that results in swelling of entire digit from synovitis and enthesitis is seen, also
called sausage digit
Enteropathic arthritis: Develops in 20% patients with inflammatory bowel disease (Crohns
or Ulcerative colitis)
Erythema nodosum and pyoderma gangrenosum can be seen
Psoriatic arthritis: Present wit oligo or mono arthritis
DIP joints are commonly involved
Arthritis mutilans wherein a pencil in cup deformity is noted on X-rays is seen in 1 to 5%
individuals
Dactylitis or sausage digits, enthesitis are other findings
Nail pitting and skin psoriasis is also seen
Extra-articular manifestations such as eye involvement (iritis, episcleritis, scleritis) is
noted
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Treatment
Ankylosing spondylitis: NSAIDs reduce pain and stiffness and recent studies show that they
can prevent progression of ankylosis
DMARDs like methotrexate and sulfasalazine are also used but studies have not shown
any benefit
TNF inhibitors like etanercept, adalimumab, and infliximab have shown promising
results
Physical therapy to improve range of motion
Reactive arthritis: NSAIDs is mainstay of treatment
Antibiotics should be used for active infection
Steroids have not shown to provide any benefit
Enteropathic arthritis: Treatment of underlying inflammatory bowel disease results in
improvement of arthritis
Psoriatic arthritis: NSAIDs help decrease pain
DMARDs like methotrexate, leflunomide, or sulfasalazine can be tried
Biologics like etanercept, adalimumab, infliximab, and golimumab have shown
promising results
Epidemiology
Primarily a disease of young women
Peak incidence between 20 to 40
Female:Male = 6-10:1
Risk Factors
Common in African Americans
Environmental trigger like exposure to ultraviolet light
Association with HLA-DR2, DR3
Clinical Manifestations
Cutaneous Involvement
Photosensitive skin rash
Malar rash on cheeks, nose, sparing nasolabial fold
Discoid rash that heals with hyper or hypo pigmentation and causes scarring
Other skin manifestations include livedo reticularis, lupus panniculitis also called lupus
profundus
Oral and/or nasal ulcers that are generally painless
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A thorough history and physical exam is needed in anyone suspected of having SLE
ANA, dsDNA, anti-Smith antibodies are tested
CBC, creatinine, and BUN levels are checked
Urine analysis looking for casts, proteinuria is checked
A spot urine protein to creatinine ratio is checked to determine degree of proteinuria
Differential Diagnosis
Other rheumatic disorders like rheumatoid arthritis, myositis, fibromyalgia
Viral infections can mimic lupus
Drug induced lupus should also be in differential (drugs like hydralazine, isoniazid,
minocycline have been implicated)
Treatment
NSAIDs
Used for pain relief especially from arthralgia/arthritis, myalgias, and headaches
In patients with kidney involvement, they should be avoided
Aseptic meningitis can be seen in some circumstances
Hydroxychloroquine
Antimalarial agent
Most frequently prescribed for lupus
First-line treatment option especially in mild disease (cutaneous, musculoskeletal
involvement)
Starting dose 200 mg daily, which is increased to 400 mg daily
Slow response taking 6 to 8 weeks
Eye exam at baseline and at 6-12 m period due to macular toxicity from cumulative dose
Corticosteroids
Provide immediate relief of symptoms
Smaller doses (5-30 mg daily) are effective in treating mild-moderate manifestations of
lupus such as arthritis, skin manifestations
Higher doses 1 mg/kg body weight are needed for severe manifestations like nephritis,
CNS manifestations, hematological manifestations
Intravenous pulse methylprednisolone can be given for life threatening manifestations (1 g
every 24 hr for 3 d)
Immunosuppressive Agents
Azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophosphamide,
leflunomide, and belimumab are other drugs used
Systemic Lupus Erythematosus and Pregnancy
No effect on disease activity
Lupus patients that have positive Ro antibody are at risk for complete heart block in the
fetus, neonatal lupus rash is also seen in newborns (it is transient and resolves without
scarring)
Thrombocytopenia is a common hematological finding in pregnant women
Presence of antiphospholipid antibodies and lupus anticoagulant can result in pregnancy
loss, preeclampsia, placental insufficiency
Epidemiology
Annual incidence in the US of about 20 cases per 1 million adults
Women are 4 times more likely to develop systemic scleroderma
African Americans are at greater risk
Presents in 3rd and 4th decades of life
10-year survival rate is 70 to 80%
Classification
Limited scleroderma: Raynauds phenomenon, GERD, swelling, and puffiness of fingers
Can be initial manifestation
Later skin thickening and tethering occurs
Anti-centromere antibody present
CREST syndrome is seen in a subgroup of patients with limited disease: Calcinosis,
Raynauds, esophageal dysmotility, sclerodactyly and telangiectasias
Pulmonary hypertension is seen as a complication in limited scleroderma
Diffuse cutaneous systemic sclerosis: Raynauds phenomenon, systemic symptoms like
malaise, arthralgias, weigh loss, skin findings (swelling then thickening and tethering, which
is more widespread)
Internal organ involvement (lung, scleroderma renal crisis, HTN, tendon friction rubs)
RNA polymerase antibody and anti-scl-70 seen in diffuse scleroderma
ILD is commonly seen in diffuse scleroderma
Systemic sclerosis sine scleroderma: Internal organ manifestations without skin thickening
Localized scleroderma: En coup de saber (linear scleroderma)
Clinical Findings
Cutaneous involvement: Telangiectasias, digital ulceration, nailfold capillary abnormalities,
skin thickening and tethering, calcinosis, salt pepper pigmentation of skin (hypo/
hyperpigmentation areas), reduced oral aperture, sclerodactyly
Vascular involvement: Raynauds phenomenon
Musculoskeletal involvement: Muscle contractures (prayer sign), tendon friction rubs,
arthralgias, myalgias, can have myositis if they have overlap syndrome
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Gastrointestinal involvement: GERD, watermelon stomach, bacterial overgrowth,
malabsorption, pseudo-obstruction
Pulmonary involvement: ILD, pulmonary HTN
Cardiac involvement: Myocardial fibrosis resulting in conduction defects, CHF
Renal involvement: Renal crisis
Diagnosis
Based on history and clinical exam (findings outlined in earlier section)
Presence of ANA, anti-centromere, RNA-polymerase and anti-scl-70 antibodies
Epidemiology
Women are commonly affected >90%
Mean age of onset 40 to 50 years
Clinical Manifestations
Sicca symptoms: Dry eyes (xerophthalmia) and dry mouth (xerostomia)
Lack of salivary secretions result in tooth decay, periodontal disease
Salivary gland swelling (parotid and submandibular glands)
Dryness of the vagina resulting in dyspareunia
Arthralgias, myalgias
Leukocytoclastic vasculitis (small vessel vasculitis) maybe seen
Raynauds phenomenon
Diagnosis
New criteria for diagnosis requires at least 2 of the 3:
Positive Ro or La antibody or positive RF and ANA (>1:320)
Positive lip biopsy showing focal lymphocytic sialadenitis
Ocular staining score 3 for keratoconjunctivitis sicca
Treatment
Symptomatic: Use of artificial tears for dry eyes
Pilocarpine and cevimeline are approved for use for dry mouth
Recent studies have shown a potential role for hydroxychloroquine
Diagnosis
Presence of anti-RNP antibody
Treatment
Corticosteroids are usually used
Mild cases with arthralgias or arthritis can be treated with aspirin or other nonsteroidal anti-
inflammatory drugs
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Clinical Manifestations
Heralded by rapid onset of pain, redness, swelling and warmth of affected joint/joints
First attack usually involves 1st metatarsophalangeal joint (podagra), gout can then
progress to involve midfoot, ankle, knee.
Joints of upper extremity occur later in the course of the disease
Diagnosis and Evaluation
Hyperuricemia; however, during an acute attack uric acid levels may fall due to deposition
in joints
Definitive diagnosis is made by synovial fluid aspiration and analysis, which shows
leukocytosis with predominance of neutrophils (>15,000 cells) and needle shaped crystals
that demonstrate negative birefringence under polarized light (yellow when light is
parallel to crystals and blue when perpendicular)
Presence of tophaceous deposits around olecranon, pinna
Treatment and Prevention
Aimed at pain relief and resolution of attack: NSAIDs, colchicine, or steroids can be used
Preventing future attacks: Colchicine and uric acid lowering medications (allopurinol and
febuxostat both are xanthine oxidase inhibitors), more recently pegloticase has been
approved for lowering uric acid levels. Drugs that cause increased secretion of uric acid in
urine such as probenecid and sulfinpyrazone can also be tried
Goal of treatment is to lower uric acid to <5 mg/dl
Dietary changes such as avoiding alcohol especially beer, purine rich foods like red meat,
shellfish, lentils, and beans. Increase dairy, fruits, and vegetable intake in diet
Physical Examination
Warmth, swelling, and tenderness of affected joint
Inability to move affected joint
Redness of overlying skin
Laboratory Studies
Peripheral leukocytosis
Elevated ESR/CRP
About 50% have bacteremia - can have positive blood cultures
Aspiration of affected joint reveals turbid or purulent synovial fluid with leukocytosis (WBC
>50,000/mm3), majority are neutrophils, gram stain is positive and so are cultures
Risk Factors
Rheumatoid arthritis
Prosthetic joints
Injection drug use
Crystalline arthritis
HIV infection
Sexual activity (predisposes to gonococcal infection)
Systemic disease (e.g., SLE, DM)
Extremes of age (<5, >65)
Common Causes
Infection With Gram-Positive Organisms
Staphylococcus and Streptococcus are common causes
Staph is most common pathogen in native joints
MRSA infections are on the rise
Approximately 70% of infections are from Staph
Vancomycin is generally used given rates of MRSA infection
3rd generation cephalosporins can also be used
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Infection With Gram-Negative Organisms
About 20% infection are from gram negative
Common in neonates, elderly, IV drug users and patients with comorbid conditions like
cancer, DM, transplant recipients
Aminoglycoside + ticarcillin or piperacillin
Disseminated Gonococcal Infection
Seen in a sexually active young person
Triad of polyarthritis, tenosynovitis and dermatitis
Synovial fluid cultures are negative
Most common presentation is of oligoarticular arthritis
Skin lesions are seen in 2/3rds and are pustular in nature
Neisseria gonorrhoeae can be cultured from the skin lesions
Urethral, cervical, rectal, pharyngeal cultures are positive in 70 to 90% cases
3rd generation cephalosporin like ceftriaxone are commonly used
Other options are fluoroquinolones
Infection With Brucella
Risk factors: Ingestion of unpasteurized milk and milk products, occupational exposure
(farmers, meat packers)
Can cause monarthritis or oligoarthritis
Sacroiliitis is also seen
Brucella melitensis is common pathogen
Prolonged course of antibiotics is needed to treat infection
Prosthetic Joint Infections
<4 weeks after surgery are due to hematogenous spread
>4 weeks after surgery are from low virulence organisms (staphylococcus epidermis)
Treatment requires antibiotics for at least 6 weeks and removal of infected prosthesis,
placement of a temporary spacer and revision arthroplasty after infection has resolved
All Causes of Septic Arthritis
All require drainage of affected joints (open surgical drainage and debridement)
Lyme Disease
Caused by Borrelia burgdorferi
Early disease presents as skin rash called erythema migrans
Can have systemic symptoms like malaise, fevers, and arthralgias
Early disseminated disease that occurs weeks to months after tick bite presents with
erythema migrans lesions, fevers, acute arthritis, carditis that manifests as AV block and
neurological manifestations of cranial nerve palsies and meningitis
Late disease occurs months to years after tick bite and presents with neurological
features (neuropathies, encephalopathy) and arthritis (usually monoarticular)
Measuring antibodies to Borrelia using ELISA (IgM 2/3 bands, IgG5/10 bands)
Goal of antibiotic therapy is to hasten resolution of signs and symptoms and prevent late
manifestations
Antibiotics that can be used are: Doxycycline, amoxicillin, and ceftriaxone is an IV option
Classification
Polymyositis
Dermatomyositis
Inclusion body myositis
Myositis associated with malignancy
Necrotizing autoimmune myositis (NAM): From medications like statins
Clinical Manifestations
Muscle Involvement
Proximal, symmetrical muscle weakness
Pharyngeal muscle weakness can result in dysphagia and dysphonia
Cutaneous Involvement
Gottron papules over MCP and interphalangeal joints
Shawl sign: Erythema over posterior shoulders and neck
V-sign: Erythema over anterior chest and upper neck
Heliotrope rash over eyelids
Nailfold capillary changes
Mechanics hands: Fissuring of lateral and palmar aspect of hands
Calcinosis is seen in juvenile dermatomyositis
Cuticular overgrowth, periungual erythema
Cardiopulmonary Involvement
Lung involvement from ILD results in Velcro like crackles
Cardiac involvement may result in EKG abnormalities, cardiomyopathy and CHF
Additional Systemic Manifestations
Myalgias, arthralgias may occur
Diagnosis
Laboratory Studies
Elevated CPK, aldolase, AST, ALT
May have elevate ESR/CRP
Specific antibodies: Anti-jo1, anti-SRP, anti-Mi2
Imaging Studies
MRI to detect muscle inflammation
Electromyography
Increased insertional activity, fibrillations, spontaneous high frequency discharges,
polyphasic motor unit potentials
Muscle Biopsy
Most useful in making a diagnosis
Perivascular inflammatory infiltrate seen in dermatomyositis
Endomysial inflammatory infiltrate in polymyositis and inclusion body myositis
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Intracellular red rimmed vacuoles seen in inclusion body myositis
Muscle fibers in various stages of necrosis and regeneration
Treatment
Steroids are first-line therapy, can start at 1 mg/kg/d
Can be given IV in severe cases
Methotrexate or azathioprine are used if patients do not respond to steroids or as steroid
sparing agents
IVIG has been tried
Other treatment options include rituximab and cyclophosphamide
Malignancy
All newly diagnosed myositis cases should have a through work-up to rule out any
underlying malignancy. Age appropriate malignancy workup (mammogram, colonoscopy),
CT scan of chest, abdomen and pelvis is also generally done
The 1st 5 years after DM diagnosis is when the risk for malignancy is greatest
Small-vessel Vasculitis
Wegener Granulomatosis
90% have nasal involvement as 1st manifestation of the disease, rhinorrhea, epistaxis,
bloody nasal crusts, cartilage inflammation may result in perforation, bony erosions of
sinus cavities
Conductive and sensorineural deafness, granulomatous inflammation of middle ear can
result in serous otitis media and compression of 7th cranial nerve
Orbital pseudotumor may result in proptosis and visual loss
Episcleritis, keratitis, uveitis
Intense inflammation of the gums (strawberry gums)
Pulmonary symptoms include cough, hemoptysis, shortness of breath, nodules, fleeting
pulmonary infiltrates, cavitary lesions in the lungs
Rapidly progressive glomerulonephritis
Arthritis
Skin involvement-digital ischemia, palpable purpura, nodules on extensor surfaces
Nonspecific symptoms like fevers, malaise, chills, weight loss
Elevated ESR/CRP, anemia, urine analysis shows RBC casts and proteinuria
Positive c-ANCA/pr-3-ANCA
Biopsygold standard in diagnosis: Lungs, kidneys, and upper respiratory tract
Treatment involves use of high dose steroids and cyclophosphamide
Cellcept or azathioprine can be used once remission is induced (for maintaining
remission)
All should receive PCP prophylaxis
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Microscopic Polyangiitis
Nonspecific constitutional symptoms fevers, chills, arthralgias, malaise, myalgias
Rapidly progressive glomerulonephritis
Mononeuritis multiplex
Cutaneous involvement-palpable purpura, ulcers, nodules, livedo reticularis
Pulmonary involvement in the form of alveolar hemorrhage, infiltrates, effusions and
fibrosis can be seen
Elevated ESR/CRP
Positive p-ANCA/MPO type
Nerve conductions studies may reveal nerve involvement and sural nerve can be biopsied
for tissue diagnosis
Renal biopsy can be done and shows glomerulonephritis
Treatment is with a combination of prednisone and cyclophosphamide
Following induction of remission, azathioprine, or methotrexate can be used in
maintaining remission
Churg-Strauss Syndrome
3 phases of the disease are seen:
1. Prodrome phase-asthma, allergic rhinitis
2. Tissue infiltrate with eosinophils
3. Vasculitis phase-organ involvement
Asthma, eosinophilia, neuropathy, pulmonary infiltrates, sinus involvement is seen
Skin involvement in the form of palpable purpura, ulcers, nodules
Cardiac involvement manifesting as CHF is seen
Glomerulonephritis can be seen
Arthralgias and arthritis is also seen
Eosinophilia, elevated IgE, positive ANCA (either MPO or pr-3 type)
Steroids are first-line and are usually effective in controlling the disease
Cyclophosphamide can be used when glomerulonephritis or neuropathy is a
manifestation
Henoch-Schonlein Purpura
Acute onset fever, palpable purpura on lower extremities, abdominal pain
(intussusception in children, intestinal angina), hematuria and arthritis are seen
Age of onset <20
Diagnosis is confirmed on biopsy that shows IgA deposition
Elevated acute phase reactants
Elevated serum IgA
Symptomatic treatment with acetaminophen, aspirin, or NSAIDs
Resolves spontaneously in about 8 weeks
Steroids can be used if there are GI symptoms and renal involvement
Cryoglobulinemic Vasculitis
Skin involvement-livedo reticularis, palpable purpura
Arthralgias are prominent
Raynauds phenomenon
Peripheral neuropathy
Membranoproliferative glomerulonephritis can be seen
90% cases have concomitant hepatitis C
Behet's Disease
Clinical Manifestations
Oral ulceration is a hallmark
Oral ulcers are painful
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Genital ulcerations can also be seen
Skin manifestations include erythema nodosum, acneiform lesions
Pathergy phenomenon: Developing a nodule or ulcer 24 to 48-hours after a needle prick
to the forearm is seen
Ocular manifestation is noted: Includes anterior or posterior uveitis
Gastrointestinal manifestations are in form of aphthous ulcers in the GI tract
Non deforming arthritis is also noted
CNS involvement is seen in 10 to 20% patients and includes headaches, aseptic meningitis,
meningoencephalitis
Thrombophlebitis, vascular thrombosis can be seen
Large vessel vasculitis can be seen in rare cases
Diagnosis
Criteria for diagnosis includes recurrent oral ulceration that occurred at least 3 times over
a 12 month period plus 2 of the following:
1. Recurrent genital ulceration
2. Ocular involvement observed by an ophthalmologist
3. Skin lesions
4. Positive pathergy test
Treatment
Steroids: Mainstay
Colchicine can be used for recurrent ulcerations and as a steroid-sparing agent
Other immunosuppressive agents used are: Dapsone, thalidomide, methotrexate, and
cyclosporine
Cyclophosphamide is used for retinal vasculitis, CNS involvement, and arteritis
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NOTES
Cheston Cunha, MD
C o n t e n t s
14.2 PHARYNGITIS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
14.3 PNEUMONIA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
14.4 ENDOCARDITIS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
14.5 HEPATITIS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
14.11 FUO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
14.13 TRANSPLANT ID . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56
14.14 ANTIBIOTICS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
Infectious Disease29
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14.1 CNS INFECTIONS
Meningitis
The classic triad is fevers, altered mental status, and stiff neck
Typical organisms in adults, in order of frequency, are S. pneumoniae, N. meningitidis,
L. monocytogenes, H. influenzae
Empiric treatment consists of a 3rd generation cephalosporin and vancomycin for resistant
S. pneumoniae
Listeria occurs in patients >60 as well as patients with underlying malignancy. If listeria is
suspected, ampicillin must be added. Use chloramphenicol or TMP-SMX if PCN allergic
Imaging of the head should be performed prior to LP in patients with HIV, evidence of
increased ICP, seizure, or focal neurologic deficits
Often, normal CSF glucose essentially rules out acute bacterial meningitis (ABM)
Cryptococcal meningitis should be considered in patients with HIV (CD4 <200), patients
with lymphoma, or acute lymphocytic leukemia (ALL)
Treat cryptococcal meningitis with amphotericin B and oral flucytosine, then will need
lifelong fluconazole
INFECTIOUS DISEASE31
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Encephalitis
Hallmark is fever, confusion/altered mental status (AMS) without stiff neck or focal
neurologic deficits
Brain Abscess
Diagnosed on CT/ MRI
Often polymicrobial and secondary to suppurative lung disease (chronic bronchiectasis),
lung abscess, right to left cardiac shunts, mastoiditis, chronic sinusitis, or acute bacterial
endocarditis (ABE)
Treat empirically with 3rd generation cephalosporin in addition to metronidazole
If open trauma/neurosurgical procedure, add MRSA coverage
Neurosyphilis
Patients often asymptomatic
Can manifest with tabes dorsalis, optic symptoms/Argyll Robertson pupil, personality
change, auditory symptoms, cranial nerve palsies
Diagnose with elevated CSF VDRL titers (only 60% sensitive) and CSF pleocytosis (>20
cells/mcL in HIV patients, >5 cells/mcL in normal host) or CSF with increased protein
CSF FTA-ABS can often be falsely positive and can only be used to rule out disease
RPR titers should decline four-fold with treatment. Failure to do so should prompt LP to
evaluate for CNS involvement
IV penicilli G is the required therapy. Those that cannot tolerate it should be desensitized.
All other regiments are suboptimal and with high failure rates
14.2 PHARYNGITIS
Mycoplasma Pharyngitis
M. pneumoniae typically presents as acute sore throat (usually without exudate) and no
cervical lymphadenopathy
M. pneumoniae often associated with otitis or bullous myringitis
C. pneumoniae presents almost identically to M. pneumoniae, but patients will often report
laryngitis which is never present with pharyngitis caused by EBV, GAS, or M. pneumoniae
Diagnose M. pneumoniae/C. pneumoniae with elevations in their respective IgM titers
Treat M. pneumoniae/C. pneumoniae with doxycycline, respiratory quinolone, or a macrolide
Streptococcal Pharyngitis
Group A streptococcal (GAS) pharyngitis presents as acute sore throat (usually with
exudates), bilateral anterior cervical lymphadenopathy, and fever
Cough and hoarseness should essentially exclude GAS pharyngitis
Group A streptococcal pharyngitis is rare in patients over age 30
Diagnosis by throat culture (presence of many PMNs differentiates infection from simple
colonization of the pharynx), GAS probe, or elevated ASO titers
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Rapid strep antigen test (RSAT) can miss up to 25% of Group A streptococcal pharyngitis
ASO titer may take up to a week to be positive
Treat Group A streptococcal pharyngitis with amoxicillin, clindamycin, or a macrolide
Treat within 10 days of onset to prevent acute rheumatic fever
Infectious Mononucleosis
Pharyngitis as part of the infectious mononucleosis syndrome is typically caused by EBV or CMV
EBV pharyngitis typically presents as acute sore throat (with or without exudate), extreme
fatigue, and bilateral posterior cervical lymphadenopathy
Diagnose EBV mononucleosis with monospot test, confirmed by elevated EBV IgM viral
capsid antigen (VCA) titer
Can differentiate between Group A streptococcal pharyngitis and EBV pharyngitis before
titers increase (may take up to 8 weeks for monospot to become positive), using ESR and
AST/ALT both of which are elevated early in EBV mono, but are normal in GAS
Other early lab clues that make EBV mono more likely are leukopenia, lymphocytosis
(classically atypical lymphs), with or without thrombocytopenia
Hoaglands sign (puffy upper eyelids) is an early clue to EBV mono, splenomegaly is a later
finding
Membranous Pharyngitis
C. diphtheriae pharyngitis presents as a membranous pharyngitis with little or no fever
Some cases develop marked enlargement of submandibular/anterior cervical lymph nodes
(bull neck)
Diagnose C. diptheriae pharyngitis with throat culture
Treatment is centered on rapid administration of diphtheria antitoxin (DAT) with adjunctive
penicillin or PO macrolide
14.3 PNEUMONIA
Community Acquired Pneumonia (CAP)
Typical CAP presents with cough, respiratory complaints, and fever unlike acute
exacerbation of chronic bronchitis (AECB) which lacks fever and has a clear CXR
Differentiate between typical and atypical by the presence of extrapulmonary findings
Diagnose with imaging, sputum culture/Gram stain (a single organism should predominate)
Normal hosts with CAP should not present with shock. This is not the case in asplenic
patients, or those with an underlying predisposition to hemodynamic compromise.
Nursing home acquired pneumonia (NHAP) is typically caused by H. influenzae, S. pneumoniae,
M. catarrhalis, or MDR aerobic Gram negative bacilli. It can be treated as CAP with a
carbapenem, respiratory quinolone, cephalosporin, or doxycycline
INFECTIOUS DISEASE35
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Table 14.4: Typical CAP (cont.)
Organism Clinical Setting/Pearls Treatment
S. aureus In CAP, S. aureus PNA only presents concurrently with viral Linezolid, van-
(CA-MRSA or influenza pneumonia comycin, mino-
MSSA) In addition to the manifestations of acute influenza, patients cycline, or anti-
develop rapid cavitation (<72 hours) on chest imaging, and Staphylococcal
cyanosis PCN if MSSA
S. aureus is not a pathogen causing CAP in the weeks following confirmed
recovery from viral influenza
Oral anaerobes Seen in patients with aspiration pneumonia Clindamycin or
Often accompanied by S. pneumoniae, H. influenzae, and a beta-lactam
M. catarrhalis + clindamycin/
Sputum Gram stain/culture often not diagnostic, diagnose on metronidazole
clinical findings/history or ampicillin/
Classically RLL, but location depends on position of patient sulbactam or
when aspiration occurred moxifloxacin
Pneumocystis Primarily seen in HIV patients, but can also present in patients TMP-SMX plus
jiroveci (PCP) on chronic immunosuppressants prednisone for
Typically presents subacutely with fever, cough, and dyspnea severe disease.
Often will become profoundly hypoxic despite a relatively clear Atovaquone,
CXR dapsone, or
Severe disease (need for adjunctive steroids) is described as primaquine in
pO2 <70 mmHg or A-a gradient >35 on ABG sulfa allergic
patients
Atypical CAP
Non-zoonotic atypical CAP is caused by Legionella sp., Mycoplasma pneumoniae, and
Chlamydophilia (Chlamydia) pneumoniae
Zoonotic atypical CAP is caused by Chlamydophilia (Chlamydia) psittaci, Coxilla burnetii,
and Francisella tularensis
Do not consider the zoonotic CAPs unless there is a clear history of recent vector exposure
Atypical CAP with significant splenomegaly is Q fever until proven otherwise
Atypical CAPs are systemic diseases with pulmonary manifestations. Symptoms noted in
the Table on Atypical CAP are found in addition to the usual cough, fever, and respiratory
complaints that accompany both typical and atypical pneumonia
M. pneumoniae Presents with subacute onset of dry cough, low grade fever (<102 F), Doxycycline,
headache, and mild myalgias over several weeks respiratory
Can be accompanied by non-exudative pharyngitis (without hoarse- quinolone, or
ness), loose stools, or bullous myringitis macrolide
Highest incidence among young adults
Associated with E. multiforme
Can cause new onset asthma in adults
CXR shows ill-defined unilateral infiltrates without effusion
Cold agglutinins can be elevated transiently early in the disease course
Diagnose with serum IgM titers, throat culture (takes 2 to 3 weeks),
or PCR from nasopharyngeal swab or sputum if present
F. tularensis Vector is tick, mosquito, or deerfly bite or direct contact with rabbits Doxycycline,
or deer respiratory
Presents with extremely acute onset of headache, myalgias quinolone,
WBC normal or low, LFTS are normal gentamicin, or
Most common form of infection is ulceroglandular tularemia which chloramphenicol
also has prominent skin manifestations (painful purple ulcer with
adenopathy)
Causes a bloody pleural effusion
Only cause of CAP with hilar lymphadenopathy (uni or bilateral)
Diagnose with serum titers
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Viral CAP
Suspect S. aureus in patients with concurrent influenza and bacterial pneumonia. These
patients are toxic in appearance often with septic shock and marked hypoxia
In patients who recover from influenza pneumonia and then develop a bacterial pneumonia
7 to 10 days later, H. influenzae and S. pneumoniae are the likely pathogens and their course
is identical to normal hosts with uncomplicated H. influenzae or S. pneumoniae CAP
Severe acute Presents as ILI with fever, dry cough, myalgias, and diarrhea Supportive,
respiratory Classically presents with biphasic disease (influenza is not unproven
syndrome biphasic), with initial symptoms improving after several days. benefit for
(SARS) Fevers and severe dyspnea then returns 7-10 days after initial steroids
infection
CXR with bilateral interstitial findings
Diagnose with SARS serology or viral isolation
Chronic Pneumonia
Chronic pneumonias present with low grade fevers, chronic (usually non-productive) cough,
and are often accompanied by malaise, night sweats, and weight loss
Symptoms must be present for at least 4 weeks to be considered a chronic pneumonia
A PPD is considered positive in patients with HIV, have a solid organ transplant, are on
chronic immunosuppression, have evidence of old TB infection on CXR, or have recently
been in contact with someone with active TB if there is greater than 5 mm of induration
Patients with diabetes, leukemia, ESRD, residents of prisons, nursing homes and homeless
shelters, healthcare workers, and IVDA have a positive PPD if greater than 10 mm of
induration is present
Normal hosts without and TB risk factors are considered positive if there is 15 mm or
greater of induration on PPD testing
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Table 14.8: Chronic Pneumonia
Organism Clinical Setting/Pearls Treatment
M. tuberculosis Presents with fever, weight loss (with intact appetite, vs. Isoniazid, rifampin,
(reactivation TB pres- malignancy), night sweats with or without hemoptysis ethambutol, and
ents as chronic PNA, Chest imaging shows bilateral apical fibro-cavitary lesions pyrazinamide.
primary TB presents No pleural effusion in reactivation TB (acute, primary TB is (treat with all 4
as acute PNA) lower lobe and commonly has effusions) initially, pending
ESR <70 mm/hr resistance studies)
Diagnose with sputum AFB positive and T-spot positive
Histoplasmosis Clinically resembles TB, but also presents with bilateral Itraconazole
hilar adenopathy (BHA)
Patients will often present with painful tongue ulcers
Geographic distribution: Mississippi River and Ohio River Valley
Diagnose with histoplasma urinary Ag
Blastomycosis Classic ulcerative/verrucous lesions develop on face/nose Itraconazole or
Organism may also involve bone or prostate amphotericin B
Right perihilar infiltrate typically seen on chest imaging
Diagnose with tissue biopsy
Coccidiomycosis Clinical presentation features prominent arthralgias and E. Amphotericin B
nodosum
Suspect Coccidiomycosis in patients with chronic pneu-
monia and eosinophilic meningitis
Chest imaging with thin walled cavities and BHA
Geographic distribution: California and American SW
Peripheral eosinophilia common
Diagnose with tissue biopsy
Nocardia Suspect Nocardia in patients with chronic pneumonia and TMP-SMX or
CNS mass lesions minocycline
Chest imaging with dense, central lower lobe infiltrates
Organism is weakly acid fast and aerobic
When stained, organism shows Chinese character forms
Diagnose with tissue biopsy/culture
Actinomyces Imaging demonstrates peripheral infiltrates Amoxicillin or
Commonly spreads to the chest wall/ribs creating fistulas doxycycline
Classic sulfur granules seen in chest wound drainage
As opposed to nocardia, actinomyces is anaerobic and not
acid fast
Diagnose with wound drainage culture
Melioidosis Clinically resembles TB, but imaging with lower lobe (not Ceftazidime
apical) involvement
Geographic distribution: Asia (exposure often decades
previously e.g., Vietnam veteran)
Diagnose with sputum culture of Burkholderia
(Pseudomonas) pseudomallei
Lung abscess Presents following aspiration pneumonia Clindamycin
Patients often with poor dentition and foul breath
Imaging with cavitary (thick walled) lung infiltrates
Requires prolonged antibiotic therapy until radiographic
resolution (1 to 3 months typically)
Diagnose with imaging/history
Rhodococcus equi Only consider R. equi in HIV patients TMP-SMX or
Presents like reactivation TB doxycycline
Diagnose on sputum stain: Filamentous forms fragment
into cocci and bacilli
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Treat early PVE empirically with vancomycin and gentamicin. Patients will show clinical
improvement, but will not be cured without valve replacement
Late PVE is usually caused by viridans streptococci or S. epidermidis (CoNS) and presents
similarly to veridans streptococci SBE
Cultures of removed valve in late PVE may be negative, but Gram stain is always positive
Empirically treat late PVE with meropenem or with vancomycin and gentamicin
PVE caused by veridans streptococci may be cured with antibiotics alone
14.5 HEPATITIS
Acute Viral Hepatitis
Acute viral hepatitis typically presents with fever (<102 F), anorexia, and malaise often with
jaundice/dark urine. Subclinical infections do occur, recognizable only by mild transaminitis
and positive serology
Fulminant acute hepatitis is one of the few causes of transaminases greater than 1,000
Hepatitis A (HAV) and hepatitis E (HEV) viruses are transmitted via fecal-oral route and
tend to have more abrupt clinical onset that other hepatitis viruses
HAV is most common in developing countries where vaccines may not be readily available,
but outbreaks can occur in daycare centers, IVDAs, or rarely in hemophilia patients (clotting
factor contaminant)
Diagnose acute HAV infection with elevated HAV IgM
HAV is usually a self limited disease and does not progress to chronic hepatitis but may
cause fulminant hepatic failure
HEV is primarily found in Southeast/Central Asia, North Africa, the Middle East, and Central
America
Pregnant patients (3rd trimester particularly) that contract HEV can often develop
fulminant hepatitis
With the exception of pregnant females, HEV is usually a self limited disease and does not
progress to chronic hepatitis
Hepatitis B (HBV), hepatitis C (HCV), and hepatitis D (HDV) are blood-borne pathogens
Approximately 10% of acute HBV infection presents as serum sickness-like illness with
migratory polyarthritis, morbiliform or urticarial rash, glomerulonephritis, and angioedema
Hepatitis B surface antigen (HBsAg) becomes positive first in acute infection, remaining
elevated for weeks to months following infection. Elevation of antibody to hepatitis B core
antigen (Anti-HBc) IgM then becomes positive
Elevated Anti-HBc IgG can only indicate prior infection as vaccination results in HBsAg IgG
positivity
Diagnose acute HBV infection with HBsAg, HBV viral load by PCR, or hepatitis B envelope
antigen (HBeAg) IgM
Give hepatitis B immune globulin (HBIG) to newborns of mothers with positive HBsAg,
following needlestick or splash accident in non-vaccinated individuals, or to sexual contacts
of acute HBV patients who have not been vaccinated
HCV is much more common in its chronic form (55 to 85% risk of going on to have chronic
infection) and most HCV infections are asymptomatic (75%)
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E. coli
Enterotoxigenic E. coli (ETEC) typically presents as acute watery, voluminous diarrhea
without blood/mucus
ETEC is the most common cause of travelers diarrhea and can be treated with supportive
care and, if febrile, with 3 days of ciprofloxacin, levofloxacin, or azithromycin
Although most E. coli diarrhea has nonbloody stool, Enterohemorrhagic E. coli (EHEC)
type O157:H7 causes an invasive dysentery with bloody stools
E. coli O157:H7 is transmitted through undercooked beef/contaminated milk and is
associated with TTP/HUS
Do not treat E. coli O157:H7 with antibiotics as this increases the likelihood of HUS
Salmonella, Shigella, Campylobacter
Salmonella, Shigella, and Campylobacter all cause invasive, frequently bloody diarrhea
In addition to the diarrhea described above, Salmonella is often accompanied by fever
and abdominal pain
Salmonella is linked to raw dairy/contaminated eggs
S. typhi enteric fever is associated with relative bradycardia, Rose spots, headache, and
abdominal pain
There is an increased risk of Salmonella sp. disease in patients with sickle cell disease
(osteomyelitis), AIDS, leukemia, lymphoma, steroid use, or recent chemotherapy
Shigella sonnei is the primary cause of bacillary dysentery in the US
S. sonnei causes an invasive, bloody diarrhea and is typically spread via fecal oral route
and can be spread from person to person particularly in areas with poor hygiene
Treat Shigella sp. with ampicillin, TMP-SMX, or a quinolone
C. jejuni is transmitted via undercooked poultry
Campylobacter infection can lead to Guillain-Barre syndrome in up to 30% of cases
following the diarrheal illness (predilection for HLA-B27 positivity)
Treat Campylobacter with azithromycin or a quinolone
Amebic Dysentery
Entamoeba histolytica presents as subacute onset of bloody stools, often with mucus, but
can also cause a more chronic dysentery
May form collar-stud abscesses in the colon
Diagnose by stool or intestinal biopsy showing organism/trophozoites
Treat with metronidazole
Viral Diarrheas
Noroviruses typically cause outbreaks of profuse watery diarrhea in the winter months
and is water-borne (can be associated with raw shellfish)
Rotavirus is typically associated with contact with children (fecal-oral) and presents as a
self-limited watery diarrhea
Diagnosis of viral pathogens is based on history/exposure but can be confirmed with
ELISA
CMV is a cause of watery diarrhea and should be suspected primarily in patients on
systemic immunosuppression
To confirm CMV infection, a colonoscopy must be performed to look for CMV inclusion
bodies
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14.8 SEXUALLY TRANSMITTED DISEASES
Urethritis
Manifests with a purulent urethral discharge. Differentiate gonococcal urethritis (GCU) from
nongonococcal (NGU) with gram stain and culture of the discharge. Discharge in NGU is
typically mucoid
GCU commonly occurs within 5 days of exposure and is accompanied by dysuria. It is
asymptomatic in 10% of males and 70% of females
Diagnose by seeing gram negative diplococci on Gram stain of urethral discharge in males,
use DNA probe or culture in females
N. gonorrhoeae infection can also involve the rectum, pharynx, and conjunctiva
Treat GCU with a single dose of 3rd generation cephalosporin or azithromycin. If no
response, consider NGU as diagnosis
NGU caused by Chlamydia trachomatis and less commonly Ureaplasma urealyticum,
Mycoplasma genitalium, or Trichomonas vaginalis
Diagnose NGU with culture of organism or DNA probe from cervical/urethral discharge.
Trichomonas can be identified on wet mount, and classically presents with strawberry
cervix
Treat all NGU except T. vaginalis with azithromycin x1 dose or doxycycline, quinolone, or
erythromycin for 1 week
Trichomonas should be treated with 2 gm metronidazole x1 dose or 2 gm tinidazole x1 dose.
Males are often asymptomatic and should be treated along with their partners
Ulcerative STDs
The chancre of primary syphilis resolves if untreated in approximately 3 to 6 weeks in a
normal host
Diagnosis based on dark field microscopy of chancre. VDRL/RPR titers take at least 1 week
to become positive, so a negative non-treponemal test does not exclude disease early on
Secondary syphilis is the result of disseminated T. pallidum. Although its manifestations are
myriad, its rash commonly involves the palms and soles and is almost always symmetric. It
may involve the oral/genital mucous membranes
Secondary syphilis is typically associated with generalized lymphadenopathy but can also
manifest as uveitis, condyloma lata, alopecia, etc.
Look for secondary syphilis 4 to 10 weeks following the initial ulcer and expect it to resolve
spontaneously 3 to 12 weeks after onset of symptoms
VDRL/RPR titers should be markedly elevated in secondary syphilis, aiding diagnosis, and
should be treated identically to primary syphilis
Latent syphilis is an asymptomatic state with a history of prior infection and elevated RPR/
VDRL. Early latent is <1 year from initial infection, late latent is >1 year or unknown
Treponemal tests will typically remain positive throughout the patients life even with
treatment, while non-treponemal titers should decrease by four-fold, or become completely
negative with treatment
Penicillin is always the preferred therapy for syphilis
Human papilloma virus Single or multiple verrucous papules on the ano- Prevent with
(HPV) genital region vaccination.
Types 16, 18, 31, 33, 35 high risk for cancer Topical therapy
Vaccine protects against types 6, 11, 16, 18 with cryotherapy/
Typically poor response to therapy laser/surgery/
imiquimod
PID/Salpingitis
Caused by N. gonorrhoeae, C. trachomatis, B. fragilis, or Enterobacteriaceae, and manifests
as lower abdominal pain, fever, and adnexal/cervical motion tenderness on pelvic exam
Must treat or infertility may develop along with other complications from scarring
N. gonorrhoeae and C. trachomatis PID patients can also develop perihepatitis (Fitz-Hugh-
Curtis Syndrome). PID with a hepatic rub is Fitz-Hugh-Curtis syndrome
Treat with moxifloxacin or doxycycline for 2 weeks
Imaging is required to evaluate for tuboovarian abscess which must typically be surgically
drained in addition to IV antibiotics
Epididymitis
Acute epididymitis presents as sudden onset unilateral testicular pain with fever
In young, sexually active males consider C. trachomatis, as the likely cause and treat with
doxycycline or levofloxacin
In older males P. aeruginosa, E. coli, and Enterobacteriaceae are the common pathogens
and can be treated with cefepime, meropenem, or levofloxacin
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Chronic epididymitis presents as >2 weeks of epididymoorchitis with epididymal nodules
Mycobacterium tuberculosis and Blastomyces dermatidis are the most common organisms
in chronic epididymitis
Treat epididymal TB as you would pulmonary TB
Blastomyces should be treated with itraconazole or amphotericin B
Vaginitis
Bacterial vaginitis/vaginosis (BV) presents as a non-pruritic vaginal discharge with a fishy
odor
It is often polymicrobial (Gardnerella vagnialis, Mobiluncus, Prevotella, Mycoplasma hominis)
Diagnose with a positive whiff test, vaginal pH <4.5, and by visualizing clue cells on wet
mount
Treat with PO metronidazole or clindamycin for 7 days
Always treat pregnant females, since BV can result in premature rupture of membranes and
premature delivery. There is an increased risk of PID in non-pregnant females with BV
Partners do not need to be treated
Candidal vaginitis manifests as pruritic white plaques in the vagina often with an
erythematous base
Diagnose with KOH prep or culture and treat with a single dose of PO fluconazole or treat
with azole vaginal creams
HIV
General Principles
Acute HIV (primary HIV) infection presents as a flu-like illness 1 to 4 weeks following
inoculation that can mimic infectious mononucleosis
Primary HIV manifests as fever, generalized lymphadenopathy, non-exudative pharyngitis,
arthralgias or myalgias, and a morbilliform rash usually on the face/trunk but may involve
the palms/soles as well as the mucous membranes
Laboratory studies initially show lymphopenia followed by a lymphocytosis (no atypical
lymphs unlike mono), and thrombocytopenia, leukopenia, and mild elevation of the
transaminases may also be present
Diagnosis of acute HIV is by HIV RNA (>20,000 copies) as it takes up to 6 months (but
usually takes 6 to 8 weeks) for HIV antibodies to rise (seroconversion)
Patients with acute HIV will still need follow-up antibody testing to confirm diagnosis
Diagnose chronic HIV with ELISA screen and confirm with Western Blot
Unless acute HIV is suspected, a negative ELISA effectively rules out HIV infection
An indeterminate Western Blot may be seen in patients undergoing seroconversion,
patients with autoantibodies related to collagen vascular disease, or with HIV-2 infection.
A HIV RNA should be obtained to exclude seroconversion, and ELISA/Western Blot
testing should be repeated in several months
Rapid HIV testing is comparable to traditional ELISA/Western Blot accuracy, but must
always be confirmed by ELISA/Western Blot testing
Once HIV infection is confirmed, all patients should undergo testing including HIV viral
load, CD4 count, lipid profile, Toxoplasma IgG, RPR/VDRL, viral hepatitis serology, and
PPD (If >5 mm induration, and CXR without evidence of active TB they should be given 9
months of isoniazid for presumed latent TB infection)
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MAC typically manifests on subacute onset of fever, weight loss, and night sweats. Focal
disease can develop once ART is initiated as a result of IRIS
Toxoplasma gondii (toxoplasmosis) almost always presents as fever and headache. It
can be accompanied by confusion, seizures, and ataxia. Causes multiple ring enhancing
lesions on MRI (CNS lymphoma is ring enhancing but is usually a single lesion). Serum
toxoplasma titers and imaging provide diagnosis. Treat with pyrimethamine, sulfadiazine,
and leucovorin (not folic acid) for 6 weeks
Important Drug Concepts and Side Effects in HIV
Simvastatin and lovastatin are contraindicated with protease inhibitorspravastatin is
preferred in these patients
Severe, life threatening lactic acidosis can develop in patients taking NRTIs for prolonged
periods (particularly stavudine [d4T], zidovudine, or didanosine)
Efavirenz is teratogenic and should be avoided in pregnant patients. Use zidovudine (pre
and intrapartum) to prevent vertical transmission and breast feeding should be avoided
Indinavir is associated with crystalluria and hematuria
Abacavir is associated with a hypersensitivity reaction (only in HLA-B5701 positive
patients) manifesting with fever, systemic rash, malaise, abdominal pain, diarrhea, nausea,
and vomiting. Typically occurs within 10 days of initiating abacavir therapy
Stavudine and didanosine can cause often irreversible peripheral neuropathy or
pancreatitis
Zidovudine is associated with bone marrow suppression and myopathy
All protease inhibitors are associated with insulin resistance and hyperlipidemia
Cutaneous Mucormycosis
Rhinocerebral mucor is seen primarily in poorly controlled diabetics. A black eschar is often
seen on the mucous membrane (nasal or hard palate)
In immunosuppressed or leukopenic hosts, cutaneous mucor presents as a black eschar on
the skin as a result of vascular invasion
Tissue stain (not culture) demonstrates the diagnostic broad, non-septate, ribbon like
branching hyphae
Treat with amphotericin B or itraconazole. Will often need surgical debridement
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Bites
Animal bites cause infection with P. multocida, GAS, Capnocytophaga canimorus (DF2), and
S. aureus. Treat with ertapenem, ampicillin/sulbactam, doxycycline, or amoxicillin/clavulanic
acid
Capnocytophaga canimorus (DF2) causes sepsis and DIC in asplenic patients bitten by cats
and dogs
Human bite wounds typically involve oral anaerobes, GAS, E. corrodens, and S. aureus.
Antibiotic therapy is identical to animal wound bites
Avoid primary closure of human bite wounds
If bitten by an animal suspected of being rabid, begin vaccination sequence immediately
(raccoon, skunk, bat, and fox bites are assumed to be rabid). If the animal is visibly rabid or
becomes rabid, give human rabies immune globulin (HRIG). If the animal can be watched
following a bite, it is possible to hold off on administration of HRIG/vaccination
Osteomyelitis
Acute osteomyelitis presents with tenderness over the infected bone, often with fever,
bacteremia, and leukocytosis. Bone scan or MRI will diagnose acute osteomyelitis in
conjunction with an ESR (often >100)
Debridement or bone biopsy is not needed for acute osteomyelitis and cure is possible with
4 to 6 weeks of antibiotics alone
Typical acute osteomyelitis organisms are S. aureus (treat with cephalosporin,
quinolone, or clindamycin if MSSA or linezolid, vancomycin, or minocycline if MRSA) and
Enterobacteriaceae (treat with cephalosporin or quinolone)
Chronic osteomyelitis typically occurs in diabetics or PVD patients and manifests as a deep
penetrating ulcer (sometimes with draining tract), normal WBC, and normal/low grade
(<102 F) fever
Plain films and ESR are sufficient for diagnosis, but MRI can also detect chronic
osteomyelitis. To isolate the causative organism, bone biopsy is the best test as blood
cultures are often negative and wound/ulcer cultures show superficial flora
P. aeruginosa is often cultured from the wound but is rarely a true pathogen unless history
of penetrating trauma through a humid, wet environment (stepping on a nail through the
bottom of a sneaker)
Definitive cure relies on surgical debridement, but can treat empirically with carbapenem,
piperacillin/tazobactam, or moxifloxacin. Use linezolid or minocycline for MRSA
Foul discharge from ulcer suggestive of B. fragilis infection requiring moxifloxacin or
ertapenem
Osteomyelitis is usually caused by Salmonella sp. in Sickle cell patients
Mycobacterium ulcerans
M. ulcerans begins as a firm, painless, mobile, subcutaneous nodule that evolves over 1 to 2
months into an enlarged fluctuant mass that eventually ulcerates with a undermined border
Geographic distribution: Africa primarily, but also present in Asia, Australia, and Central/
South America
Diagnose by AF stain/biopsy of ulcer
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14.11 FUO
Definition: Fever of unknown origin is defined as a temperature >101 F (38.3 C) x 3 weeks that
is undiagnosed after 3 days of hospitalization, 3 outpatient visits, or 1 week of intensive outpatient
investigation. Typically, the longer the period the fever remains undiagnosed, the less likely it is
infectious is origin.
Causes: 4 major categories: 1. Infectious 2. Malignancy 3. Rheumatologic 4. Other.
Workup: Testing should be directed at any potential clues found on thorough history and
exam. It is important to also consider the patients age when directing the workup and consid-
ering the cause, i.e., malignancy is more likely in elderly patients with FUO; GCA should not be
considered in patients less than 50; SLE or RA rare to present later in life.
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Ehrlichia chaffeensis (Human monocytic ehrlichiosis - HME)/Anaplasma (Ehrlichia)
phagocytophilium (Human granulocytic anaplasmosis - HGA)
HME vector is Ambylomma americanum (lone star tick). HGA vector is the Ixodes tick
HME/HGA presents acutely with chills, headache, malaise, myalgias, and fever
Clinically very similar to RMSF but without rash
Laboratory tests are notable for leukopenia with relative lymphopenia, thrombocytopenia,
low ESR, high ferritin, and elevated transaminases
Diagnose with serum PCR or classic mulberry shaped, basophilic morula in neutrophils
(HGA only). Otherwise use organism specific titers (no crossreactivity)
Treat HME and HGA with doxycycline
14.13 TRANSPLANT ID
General Principals
Transplant recipients can develop nosocomial/post-operative infections, reactivation of
latent infection, or infection from infected donor organs (donor derived infections)
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14.14 ANTIBIOTICS
Syndrome specific antibiotic pearls are covered in each previous section. This section is
reserved for other high-yield Board pearls and notable side effects.
Pearls
Ertapenem is the only carbapenem that does not cover Pseudomonas aeruginosa
Daptomycin should not be used for pneumonia, as it is inactivated by the calcium in
surfactant
Of the cephalosporins, only ceftazidime (3rd GC), cefoperazone (3rd GC), and cefepime
(4th GC) are active against Pseudomonas aeruginosa
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NOTES
Michael J. Sanley, MD
C o n t e n t s
15.1 ASTHMA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62
PULMONARY DISEASE61
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15.1 ASTHMA
Epidemiology
Affects roughly 300 million people worldwide
Common onset in childhood, but can have adult onset
Associated with atopy in most patients
Pathogenesis
Hallmark is chronic lower airway inflammation
Mucosal infiltration of inflammatory cells neutrophils and eosinophils
Thickening of basement membrane due to collagen deposition
Goblet cell hyperplasia leads to increased mucous production and plugging
Diagnostic Testing
Pulmonary function testing
Reduced FEV1, FEV1/FVC ratio, peak expiratory flow
Significant response to bronchodilator is an increase in FEV1 or FVC by 200 mL or
more or greater than or equal to 12%
Methacholine challenge decrease of FEV1 by 20% deemed positive for airway reactivity
Can be helpful in determining asthma as cause of chronic cough
Exercise-induced Bronchospasm
Onset typically after exercise with resolution usually by 30 minutes
Treated with short acting b2-agonists prior to exercise
PULMONARY DISEASE63
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Management
Table 15.1: Assessment of Asthma Control
Classification of Asthma Severity
>12 Years of Age
Components of Severity
Persistent
Intermittent Mild Moderate Severe
Symptoms 2 days/week >2 days/ Daily Throughout
week but not the day
daily
Nighttime 2x/month 3-4x/month 1x/week but Often 7x/
awakenings not nightly week
Short-acting 2 days/week >2 days/ Daily Several times
b2-agonist use week but not per day
Impairment daily, and not
for symptom
control (not more than 1x
Normal on any day
FEV1/FVC: prevention of
8-19 yr 85% EIB)
20-39 yr 80% Interference None Minor Some Extremely
40-59 yr 75% with normal limitation limitation limited
60-8 yr 70% activity
Lung function Normal FEV1 FEV1 >80% FEV1 >60% FEV1 <60%
between exac- predicted but <80% predicted
erbations FEV1/FVC predicted FEV1/FVC
FEV1 >80% normal FEV1/FVC reduced
predicted reduced 5% >5%
FEV1/FVC
normal
Risk Exacerbations 0-1/year 2/year
requiring oral
systemic Consider severity and interval since last exacerbation.
corticosteroids Frequency and severity may fluctuate over time for patients in any
severity category.
Pharmacotherapy
Bronchodilators
b2-agonists relaxes airway smooth muscle, inhibits mast cell mediator release, and
airway edema. Improves mucociliary clearance. Decreases cough. Does not decrease
chronic inflammation
Short acting b2-agonists: Albuterol, levalbuterol, terbutaline
Long acting b2-agonists: Salmeterol, formoterol
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Table 15.2: Stepwise Approach to Asthma Therapy
Persistent Asthma: Daily Medication
Intermittent
Consult with asthma specialist if Step 4 care or higher is required.
Asthma
Consider consultation at Step 3.
Step 6
National Heart, Lung, and Blood Institute. National Asthma Education and Prevention Program: Expert panel report III:
Guidelines for the diagnosis and management of asthma. Published Bethesda, MD;2007. Accessed June 17. 2012.
Risk Factors
#1 risk factor = smoking
Dose-response relationship the more you smoke, the higher your risk
Inhaled particles from other sources also increase risk (2nd hand smoke, wood/coal
furnaces, air pollution)
Alpha-1 antitrypsin deficiency
Pre-existing airway responsiveness (e.g., asthma)
Recurrent severe respiratory infections in childhood causing decreased lung function
Symptoms
Chronic cough
Dyspnea
Sputum production
Physical Findings
Signs of airflow limitation usually in more severe disease
Diagnosis
Spirometry: FEV1/FVC <70% predicted POST bronchodilator is diagnostic
Severity
GOLD 1 MILD: FEV1 greater than or equal to 80% predicted
GOLD 2 MODERATE: FEV1 between 50% and 80% predicted
GOLD 3 SEVERE: FEV1 between 30% and 50% predicted
GOLD 4 VERY SEVERE: FEV1 <30% predicted
Treatment
Smoking cessation
Stable disease
Bronchodilators
b2-agonists: Improves FEV1 and symptoms. Long acting more effective at
preventing symptoms
Anticholinergics improve symptoms, exacerbations, pulmonary rehab
Theophylline improves symptoms and exacerbations
Inhaled corticosteroids
Improves lung function, symptoms, quality of life, and exacerbations
More effective when combined with a bronchodilator
Pulmonary rehabilitation
Improves exercise capacity, perceived breathlessness, quality of life, decreases days
in hospital
Oxygen therapy
Indicated for resting SaO2 <88% at 2 visits over 3 weeks
Improves survival in patients who meet criteria
Lung volume reduction therapy
Improves survival in patients with severe upper lobe emphysema
Lung transplant
Indicated for very severe COPD with FEV1 <20%
Improves quality of life and functional capacity
Exacerbation acute change in dyspnea, cough or sputum production
Indications for hospitalization: Drastic change in symptoms, older age, frequent
exacerbations, serious comorbidities, severe underlying COPD
Oxygen therapy goal SaO2 >90%
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Short acting bronchodilators
Systemic corticosteroids oral prednisone 30-40 mg daily x 14 days
Antibiotics indicated for patients with:
All 3 cardinal symptoms (dyspnea, cough, and increased sputum purulence)
2 cardinal symptoms if 1 is sputum purulence, or
Patient requires mechanical ventilation
Mechanical ventilation
Non-invasive positive pressure ventilation (NIPPV): Reduces mortality, need for
endotracheal intubation and length of hospital stay
Invasive mechanical ventilation: Indicated for failure of NIPPV, respiratory or cardiac
arrest, altered mental status, aspiration, life-threatening hypoxemia, hemodynamic
instability
Discharge and follow-up
Criteria for discharge:
Decreased need for short acting bronchodilators less than every 4 hours
Ability to ambulate, eat, sleep without significant dyspnea
Clinically stable for 12 to 24 hours
Arrangements should be made for home care, oxygen as needed. Follow-up visit
should be scheduled within 4 to 6 weeks
Evaluation
Age/sex of patient important helps narrow differential
Exposure history smoking, drugs, radiation, occupational exposures
Onset usually over months to years, acute presentation uncommon
Radiologic findings
High-resolution CT scan more sensitive than chest radiograph
Reticular pattern most common interlacing linear opacities
Honeycombing series of cysts with shared walls commonly seen in idiopathic
pulmonary fibrosis
Cysts enlarged airspaces surrounded by wall
Nodules round, discrete opacities
Ground-glass opacities hazy increase in attenuation but can still see vasculature
Important to note distribution diseases often involve primarily upper vs. lower lung or
central vs. peripheral lung
Histopathologic diagnosis
Surgical biopsy has 90% diagnostic yield
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Pneumothorax in 25% of patients
Radiographic findings: Stellate nodules, reticular opacities, upper zone cysts,
preserved lung volume, sparing of costophrenic angles
HRCT combination of thin walled cysts and nodules virtually diagnostic in
appropriate patient
Fibrosis develops as disease progresses
Mainstay of treatment = smoking cessation
Improvement in 1/3 of patients, most have persistent or progressive disease
Desquamative interstitial pneumonia
Exclusively in smokers
Histology: Accumulation of macrophages in intraalveolar spaces, minimal fibrosis
Symptoms: Dyspnea and cough
HRCT diffuse hazy opacities
Treatment: Smoking cessation and glucocorticoids
70% 10-year survival
Silicosis
Seen in miners, stonecutters, sandblasters
Profuse military infiltration on chest radiograph. HRCT shows characteristic crazy paving
pattern diffuse ground-glass opacities with thickened intralobar and interlobular septa
Greater risk of active tuberculosis requires longer treatment of latent TB
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15.5 PLEURAL DISEASE
Causes of Pleural Effusion
Transpleural pressure imbalance
Increased capillary permeability
Impaired lymphatic drainage
Movement of peritoneal fluid across the diaphragm
Extravascular effusion
Elevated pleural fluid amylase pancreatic vs. malignant effusion vs. esophageal rupture
Low pleural fluid glucose bacterial infection vs. malignancy vs. rheumatoid pleuritis
Cytology helpful in assessment for malignancy
Management
Parapneumonic effusions
Require thoracentesis for evaluation
Require chest tube placement if:
Loculated
Pleural fluid pH <7.2
Pleural fluid glucose <60 mg/dL
Positive gram stain or culture
Pus in pleural space (empyema)
Malignant pleural effusions
Can usually get diagnosis from cytology, but may require thoracoscopy
Symptomatic treatment with therapeutic thoracentesis
Small indwelling catheter can be inserted for repeated drainage
Consider pleurodesis to prevent re-accumulation
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Warfarin for maintenance therapy
Continue heparin until INR 2-3 (need overlap of therapies for 4 to 5 days)
Duration of therapy:
Provoked DVT/PE (e.g., trauma, surgery) = 3 to 6 months
Initial unprovoked DVT/PE = 6 months
2nd unprovoked/cancer/hypercoagulable state = lifetime
Unstable PE
Consider thrombolytics if signs of shock, hemodynamic, or respiratory compromise
WHO Classifications
Group I: Pulmonary arterial hypertension
Idiopathic, familial, HIV associated, drug induced, portal hypertension, congenital shunts
Bone morphogenic protein receptor 2 (BMPR2) gene
Mutations seen in 50% of families with FPAH
Group II: Pulmonary hypertension owing to left heart disease
Group III: Pulmonary hypertension owing to lung diseases and hypoxia
Group IV: Chronic Thromboembolic Pulmonary Hypertension
Diagnose with V/Q scan or CT angiogram
Treatment: Pulmonary thromboendarterectomy significant improvement in survival
and quality of life
Group V: Pulmonary hypertension with unclear or multifactorial etiologies
Treatment of PAH
Anticoagulants - improved survival
Oxygen
Diuretics - symptomatic improvement and treatment of right heart failure associated with
PAH
Calcium channel blockers - benefit seen only in those patients that demonstrate positive
vasoreactivity on right heart catheterization
Prostacyclins - improved clinical measurements and survival
Epoprostenol, iloprost, treprostinil
Endothelin receptor antagonists - improvement in exercise capacity, functional class,
hemodynamics, and time to clinical worsening
Bosentan, ambrisentan
Phosphodiesterase inhibitors - improved 6 minute walk test and pulmonary vascular
resistance
Sildenafil, tadalafil
Clinical Presentation
5 to 15% diagnosed incidentally with imaging
Most present with symptoms
Local tumor growth can cause:
Cough, hemoptysis, dyspnea
Pleural or chest wall pain
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Spread into thorax can cause:
Tracheal obstruction
Dysphagia due to esophageal compression
Hoarseness due to recurrent laryngeal nerve paralysis
Horners syndrome: Enophthalmos, ptosis, miosis and ipsilateral loss of sweating
Pancoasts syndrome: Tumor growth into apex of lung, affects C8-T2 nerves,
causing radiating pain down ulnar nerve
Superior vena cava syndrome
Extrathoracic metastasis can cause:
Brain metastasis: Headache, nausea, neurologic defects
Bone: Pathologic fracture
Spine: Cord compression syndrome
Liver: Abnormal LFTs, biliary obstruction, abdominal pain
Paraneoplastic syndromes
Syndrome of inappropriate anti-diuretic hormone (SIADH)
Cushing syndrome
Hypercalcemia
Clubbing
Hypertrophic osteoarthropathy
Eaton-Lambert syndrome
Migratory venous thrombophlebitis
Nonbacterial thrombotic endocarditis
Treatment
Non-small cell lung cancer
Stage I-II: Resection if candidate. If not, radiation therapy
Adjuvant chemotherapy for stage II
Stage III: Local resection or radiation with systemic chemotherapy
Stage IV: Chemotherapy for palliative and improved survival
Median survival for untreated is 4 to 6 months, for treated is 8 to 10 months
Bevacizumab improves response rate, survival when added to chemotherapy
Small cell lung cancer
Chemotherapy is mainstay of treatment 60 to 80% response rate with 10 to 30%
complete response
In limited stage disease, addition of radiation therapy improves survival
High relapse rate
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Carcinoid Tumors
Neuroendocrine tumors, often present with cough, hemoptysis or bronchial obstruction
Treatment is surgical with 90% 10-year survival post resection if no nodal involvement
Atypical carcinoid histologic evidence of increased mitosis and dysplasia worse
prognosis with 60 to 70% 5 year survival
Mediastinal Masses
Anterior Mediastinal Masses
Thymoma
Lymphoma
Teratoma
Thyroid mass
Middle Mediastinal Masses
Vascular mass
Lymph node enlargement
Pleuropericardial cyst
Bronchogenic cyst
Posterior Mediastinal Masses
Neurogenic tumor
Meningocele
Meningomyelocele
Gastroenteric cyst
Esophageal diverticula
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15.10 HIGH-ALTITUDE PULMONARY DISEASE
Acute Mountain Sickness
Onset within 6 to 10 hours of ascent to high altitude
Symptoms: Lightheadedness, nausea, headache, tingling
Treatment: Descend to lower altitude (usually enough to relieve symptoms),
dexamethasone, acetazolamide
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Weaning
Patients should undergo daily awakening and spontaneous breathing trials to facilitate
extubation
Reduces time on ventilator, ICU time, hospital length of stay and 1-year mortality
Ventilator-associated Pneumonia
Definition: Onset of pneumonia at least 48 hours after initiation of invasive ventilation
Significant mortality 25 to 50%
Important to reduce risk: Elevate head of bed, consider limiting enteral feeding early in
disease, consider selective decontamination of GI tract and subglottic suction catheter
Sepsis
Diagnosis known infection plus at least 2 systemic inflammatory response syndrome
(SIRS) criteria:
Temperature >38 C (100.4 F) or less than 36 C (96.8 F)
Leukocyte count >12,000/L or less than 4,000/L
Respiratory rate >20/min
Heart rate >90/min
Severe sepsis = sepsis + evidence of 1 sepsis-induced organ dysfunction
Septic shock = sepsis-induced hypotension, persistent despite fluid resuscitation
Early goal directed therapy within 1st 6 hours
Early antibiotic initiation
Fluid resuscitation to goal central venous pressure of 8-12 mmHg
Initiation of vasopressors as needed for goal mean arterial pressure >65 mmHg
Urine output >0.5 mL/kg/hr
Central venous oxygen concentration >70%
Achieved by transfusion if hematocrit <30% or inotropic agents (dobutamine) if
>30%
Anaphylaxis
Symptoms: Urticaria, angioedema, hoarseness, stridor, tachycardia, bradycardia (severe
reaction), hypotension, cardiovascular collapse, respiratory arrest
Treatment: Intramuscular or subcutaneous epinephrine (0.3-0.5 mg of 1:1,000)
Use IV epinephrine (1:10,000) in anaphylactic shock
Important to monitor for recurrence
Common causes: Peanut, insect stings, medications, latex exposure
Hypertensive Emergencies
Elevated blood pressure with evidence of end organ dysfunction
Dont correct blood pressure too quickly no more than 25% in 1st hour
Dropping too low can cause hypoperfusion stroke due to cerebral autoregulation
IV medications include: Hydralazine, nitroprusside, nitroglycerin, labetalol, metoprolol,
esmolol, nicardipine
Hyperthermic Emergencies
Heat stroke
Signs/symptoms: Absence of sweat, altered consciousness, muscle rigidity, seizure
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Severe cases can cause rhabdomyolysis, disseminated intravascular coagulation, ARDS
Treat with cooling blankets, ice packs, cooled IV fluids, cold gastric lavage
Malignant hyperthermia
Caused by exposure to certain anesthetics and depolarizing muscle relaxants
Halothane, isoflurane, enflurane, desflurane, sevoflurane, succinylcholine,
decamethonium
Signs/symptoms: Increased muscle rigidity, tachycardia, hypercarbia, hypertension,
tachypnea, hyperthermia, arrhythmias
Inherited disorder important to note family history
Treatment: Removal of offending agent, supportive care, dantrolene every 5 to 10
minutes until resolution of symptoms
Neuroleptic malignant syndrome
Caused by exposure to certain antipsychotics and tranquilizers
Includes: Haloperidol, chlorpromazine, fluphenazine, clozapine, risperidone,
metoclopramide
Signs/symptoms: Muscle rigidity, hyperthermia, altered mental status, seizure,
arrhythmia, sialorrhea, diaphoresis
Onset typically within 2 weeks of drug initiation
Treatment: Removal of offending drug. Dantrolene for serious reactions
Hypothermia
Associated with tachycardia followed by worsening bradycardia, conduction abnormalities,
respiratory depression, altered mental status
EKG: Osborne waves
Treatment
Active external rewarming
Active core rewarming
Passive external rewarming
Rhabdomyolysis
Breakdown of myocytes causes myoglobinuria and renal failure
Elevated CK, positive urine dipstick for blood suggests myoglobinuria
Treatment: Aggressive IV hydration, goal urine output 300 mL/hr. May require dialysis
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REFERENCES & SUGGESTED READINGS
1. Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL, Loscalzo J. (2008). Harrisons
principles of internal medicine (17th ed.). New York: McGraw-Hill Medical Publishing Division.
ISBN978-0-07-146633-2. http://www.mhprofessional.com/product.php?isbn=0071466339&cat=4.
2. Fanta CH. Asthma. N Engl J Med. 2009;360(10):1002-1014.
3. National Heart, Lung, and Blood Institute. National Asthma Education and Prevention Program:
Expert panel report III: Guidelines for the diagnosis and management of asthma. www.nhlbi.nih.gov/
guidelines/asthma/asthgdln.htm. Published Bethesda, MD;2007. Accessed June 17. 2012.
4. Hicks M, Brugman SM, Katial R. Vocal cord dysfunction/paradoxical vocal cord motion. Prim Care.
2008;35(1):81-103.
5. Global Initiative for Chronic Obstructive Lund Disease (GOLD). Global Strategy for the Diagnosis,
Management and Prevention of COPD. www.goldcopd.org. Revised 2011. Accessed June 17, 2012.
6. Ryu, JH et al. Diagnosis of interstitial lung diseases. Mayo Clin Proc. 2007;82(8):976-986.
7. Light RW. Pleural effusion. N Engl J Med. 2002;346:19717.
8. Bller HR, Agnelli G, Hull RD, et al. Antithrombotic therapy for venous thromboembolic disease:
the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy. Chest. 2004;126(3
Suppl):401S-428S.
9. Chunilal SD, Eikelboom JW, Attia J, et al. Does this patient have a pulmonary embolism? JAMA.
2003;290(21):2849-2858.
10. Simonneau G et al. Updated Clinical Classification of Pulmonary Hypertension. JACC. 2009;54:S43-
54.
11. Runo JR, Loyd JE. Primary Pulmonary Hypertension. Lancet 2003;361:1533-44.
12. Alberg AJ, Ford JG, Samet JM. American College of Chest Physicians. Epidemiology of lung cancer:
ACCP evidence-based clinical practice guidelines (2nd Edition). Chest. 2007;132( 3 Suppl):29S-55S.
13. Gould MK, Fletcher J, Iannettoni MD, et al. American College of Chest Physicians. Evaluation of
patients with pulmonary nodules: when is it lung cancer? ACCP evidence-based clinical practice
guidelines. Chest. 2007;132( 3 Suppl):108S-130S.
14. Goldstraw, P, Crowley, J, Chansky, K, et al. The IASLC Lung Cancer Staging Project: Proposals for
the revision of the TNM stage groups in the forthcoming (seventh) edition of the TNM classification
of malignant tumours. J Thorac Oncol. 2007; 2:706.
15. Pennell N. Selection of chemotherapy for patients with advanced nonsmall cell lung cancer. Cleve
Clin J Med. 2012;79 Electronic Suppl 1:eS46-50.
16. Duwe BV, Sterman DH, Musani AI. Tumors of the mediastinum. Chest. 2005;128(4):2893-909.
17. Eckert DJ, Jordan AS, Merchia P, Malhotra A. Central sleep apnea: Pathophysiology and treatment.
Chest. 2007;131(2):595-607.
18. Luks AM, Swenson ER. Medication and dosage considerations in the prophylaxis and treatment of
high-altitude illness. Chest. 2008;133(3):744-55.
19. Hackett PH, Roach RC. High-altitude illness. N Engl J Med. 2001;345:107114.
20. The Acute Respiratory Distress Syndrome Network. Ventilation with lower tidal volumes as com-
pared with traditional tidal volumes for acute lung injury and the acute respiratory distress syn-
drome. N Engl J Med.2000 May 4;342(18):1301-8.
21. Ware LB, Matthay MA. The Acute Respiratory Distress S. N Engl J Med. 2000;345(18):1334-1349.
22. Hill NS, Brennan J, Garpestad E, Nava S. Noninvasive ventilation in acute respiratory failure. Crit Care
Med. 2007;35:2402-2407.
23. Girard TD, Kress JP, Fuchs BD, et al. Efficacy and safety of a paired sedation and ventilator weaning
protocol for mechanically ventilated patients in intensive care (Awakening and Breathing Controlled
Trial): a randomised controlled trial. Lancet. 2008;371(9607):126-134.
24. Finfer S, Chittock DR, Su SY, et al. NICE-SUGAR Study Investigators. Intensive versus conventional
glucose control in critically ill patients. N Engl J Med. 2009;360(13):1283-1297.
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NOTES
C o n t e n t s
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16.1 DISORDERS OF THE ESOPHAGUS
Symptoms of Esophageal Disorders
Dysphagia
Definition of Dysphagia obstruction of passage of liquid or food through the esophagus
Classified as either oropharyngeal or esophageal
Oropharyngeal dysphagia arises from disorders that affect the function of the
oropharynx, larynx, and upper esophageal sphincter
Zenkers diverticulum typically is encountered in elderly patientsthey typically present
with regurgitation, dysphagia, and halitosis; They present with a triad of dysphagia,
regurgitation, and halitosis
Esophageal dysphagia arises within the body of the esophagus, the lower esophageal
sphincter, or cardia, and is most commonly due to mechanical causes or a motility
disturbance
The most common structural causes of dysphagia are Schatzkis rings, eosinophilic
esophagitis, and peptic strictures. Dysphagia also occurs in the setting of
gastroesophageal reflux disease without a stricture
Dysphagia for solids is usually caused by a structural lesion such as strictures, lower
esophageal ring, cancer and Plummer Vinson Syndrome: Include Plummer-Vinson
Syndrome: triad of esophageal web, iron deficiency anemia and cancer;; dysphagia for
both solids and liquids is usually caused by a motility disorder
For suspected esophageal dysphagia, endoscopy is the single most useful test.
Endoscopy allows better visualization than does barium radiography along with mucosal
biopsies, and therapeutic intervention
Videofluoroscopyis the most effective study for suspected oropharyngeal dysphagia
Noncardiac Chest Pain/Esophageal Origin
1. Pain persisting for more than 1 hour
2. Pain that typically occurs postprandially and reclining and wearing tight clothes
3. Lack of radiation of the pain
4. Associated esophageal symptoms (heartburn, regurgitation, dysphagia)
5. Pain relieved by antacid
Odynophagia and Globus Sensation
Definition: odynophagia as pain that occurs with swallowing
Odynophagia is the most common presenting symptom in infectious esophagitis, which
is most frequently caused by Candida Albicans, especially in the immunocompromised,
patients on chronic prednison
Globus sensation (also known as globus hystericus) is a feeling of a lump or tightness in
the throat; psychological factors account for most cases, GERD can be detected in many
affected patients
Sources: 1.
1. Katz PO, Gerson LB, Vela MF. Guidelines for the diagnosis and management of
gastroesophageal reflux disease. Am J Gastroenterol. 2013 Mar;108(3):308-28. [184
references]
Barrett Esophagus
Screening and Diagnosis
A complication of GERD in which normal squamous epithelium of the distal esophagus is
replaced by specialized columnar epithelium
It is a premalignant condition, affected patients have a 30-fold increased risk of
esophageal adenocarcinoma. Risk factors : age > 50 years, white, male, obesity, chronic
GERD.
Endoscopic surveillance is suggested for patients with Barretts esophagus as follows:
No dysplasia: 3 to 5 years
Low-grade dysplasia: 6 to 12 months
High-grade dysplasia in the absence of eradication therapy: 3 months
Treatment
Therapy with PPIs alone has not been shown to slow progression of Barrett
For low-grade dysplasia, radiofrequency ablation may be an appropriate therapeutic
alternative
UPDATED GUIDELINE: Endoscopic therapy with RFA, endoscopic mucosal resection
(EMR) and photodynamic therapy (PDT) should be attempted in high-grade Barretts
before surgery [1]
Esophagectomy is the only therapy for high-grade dysplasia
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position statement on the management of Barrett's esophagus. Gastroenterology. 2011
Mar;140(3):1084-91
Esophageal Carcinoma
Two main types : are squamous cell carcinoma, and adenocarcinoma. Both types are becom-
ing equally prevalent in the United States. Major Risk factors: age, sex, smoking, alcohol, chronic
GERD, Barretts, diet, Plummer Vinson Syndrome. Signs and Symptoms: dysphagia, odynophagia,
dysphonia, cough as a result of laryngeal nerve injury.
Diagnosis and Staging
The diagnosis is usually established by endoscopic biopsy
Staging with a CT scan of the chest, abdomen and pelvis with contrast to evaluate for the
presence of metastatic disease
Patients without evidence of metastatic disease should undergo endoscopic
ultrasonography (EUS)
Treatment
Depending on staging, treatment modalities differ
Stage 0-1 (localized disease : endoscopic theray with RFA, EMR, PDT)
Stages I-IV: chemotherapy as adjuvant therapy
Esophagectomy and esophageal stenting is used as a palliative care measure in late stage
Prognosis
Because cancer is caught late, there is a low 15% 5 year survival rate with most patients
dying in the first year
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Figure 16.3: Upper endoscopy showing Candida
esophagitis
Pill-induced Esophagitis
Most common medications are tetracycline, iron sulfate, bisphosphonates, potassium,
NSAIDs, and quinidine
Taking a large fluid bolus prior to swallowing pills or remaining upright for 30 minutes
after taking medication might help
Eosinophilic Esophagitis
Emerged as an important cause of dysphagia that is amenable to treatment by
elimination of dietary allergens or topical glucocorticoids
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Complications of Nonsteroidal Anti-inflammatory Drugs
Include dyspepsia, peptic ulcer disease, and strictures
Prevention and Treatment of NSAID-induced Injury
The risk of GI injury with COX-2 inhibitors is significantly lower than that of traditional
NSAIDs
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Therapy: Gluten-free diet for treatment of celiac disease or dermatitis herpetiformis (even
with the absence of GI manifestation)
Supplement deficient vitamins and nutrients
Noncompliance is the most common reason for failure and relapse
Small-intestine Bacterial Overgrowth
A condition in which colonic bacteria are present in increased numbers resulting in
excessive fermentation, inflammation, or malabsorption
Symptoms include flatulence, bloating, and constipation
May occur in association with anatomical abnormalities (surgical blind loop) or motility
disorders (scleroderma, diabetes mellitus)
The gold standard for diagnosis is an excessive bacterial growth in a jejunal aspirate; gold
standard: > 10^5 bacterial count ; normal -10^4)
D-xylose test :patient drinks D-xylose, and because it does not require enzyme for
digestion; presence in the urine and blood is a positive test for colonization of the bowel,
impairing absorption
Treatment includes antibiotics for 10 to 14 days
Short-bowel Syndrome
Malabsorptive state secondary to small bowel resection
Early management mostly involves replacement of fluid and electrolytes
Enteral feeding may be gradually introduced
Pancreatic Insufficiency
Patients with impaired pancreatic function present with symptoms of fat malabsorption;
symptoms include: steatorrhea, weight loss, anemia, hypoalbuminemia
Co-morbidities: Cystic fibrosis, inflammatory bowel disease, celiac disease.
Resolution of symptoms after an empiric trial of enzyme is often a confirmatory test
Diagnosis
Although several features may differentiate Crohn disease from ulcerative colitis, there is
significant overlap.
Treatment of Ulcerative Colitis
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Moderate to severe Prednisone: Remission induction
Infliximab: Refractory disease
Maintenance therapy: 6-mercaptopurine (6-MP), azathioprine, or methotrexate
Severe IV corticosteroids: Remission
Infliximab: Corticosteroid-refractory disease
Surgery with colectomy: Extremely toxic patient or does not respond to
medication
Diagnosis:
Colonoscopy with biopsy; CT enterography
Guidelines for surveillance colonoscopy: concensus: 8-10 years after diagnosis; then every
year after that depending on the risk
Microscopic Colitis
Includes: Lymphocytic colitis or collagenous colitis
Peak incidence at age 65
Characterized by chronic diarrhea, often with abdominal pain and mild weight loss
Colonoscopy with tissue biopsies is required for diagnosis, mucosa usually normal on
gross visualization
Dysmotility Syndromes
Constipation
Alarm features include rectal bleeding, weight loss, a family history of colon cancer or
IBD, anemia, and acute onset in elderly patients
Ileus and Pseudo-obstruction
Usually after surgery (especially abdominal) and can be associated with metabolic
disturbances (electrolyte abnormalities, sepsis); medications (anticholinergics, narcotics, etc.)
Treatment depends on symptoms and etiology
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16.4 DISORDERS OF THE PANCREAS
Acute Pancreatitis
The causes of acute pancreatitis US include:
1. Biliary obstruction and alcohol (most common about 80% of cases)
2. Sulfonamides, estrogens, didanosine (ddI), valproic acid, thiazide diuretics, and
furosemide
3. Hypertriglyceridemia (>1,000 mg/dL)
4. Endoscopic retrograde cholangiopancreatography (ERCP)
5. Cystic fibrosis (young people with pancreatitis) and pancreas divisum
Clinical Presentation and Diagnosis
Typical symptoms include: Sudden onset of epigastric pain, often radiating to the back
with nausea, vomiting, fever, and tachycardia
The serum amylase and lipase levels are elevated in 75 to 90% of patients (>3 times the
upper limit of normal)
Lipaseis preferred to amylase due to its longer half-life and increased specificity
Mildly increased amylase values can be caused by:
Renal insufficiency
Intestinal ischemia
Appendicitis
Parotitis
Prognostic Criteria for Acute Pancreatitis
Various scoring systems are used to identify at-risk patients and to determine prognosis;
these include the Ranson criteria, the Glasgow Scoring System, and the APACHE II
In the intensive care unit, the Severity of Organ Failure Assessment (SOFA) score has
been shown to be superior to the Ranson and APACHE score
On admission to the ED, 2006 guideline update has shown the APACHE score is more
sensitive to predicting mortality in acute pancreatitis
BISAP score is a reliable method in stratifying clinical severity of acute pancreatitis within
24-hours of hospital admission. It is a new scoring system that takes into consideration
the following clinical criteria:
Blood urea nitrogen level greater than 25 mg/dL
Impaired mental status
Systemic inflammatory response syndrome
Age >60 year
Pleural effusion
Management
Mild pancreatitis is treated with supportive care including pain control, intravenous fluids,
correction of electrolyte and metabolic abnormalities
Updated guidelines: use of Lactated Ringers solution is associated with reduction in
inflammation (84% vs 0%) compared to normal saline, and should be used in volume
repletion. Contrary to the belief against using opioids in causing sphincter of oddi spasms,
there is limited studies showing that morphine is a culprit; therefore, clinicians should use
fentanyl or Demerol, but should be wary of side-effects
Moderate-severe pancreatitis in addition to supportive care and intensive care unit
monitoring:
TPN or enteral jejunal feedings (preferred)
Chronic Pancreatitis
Chronic pancreatitis is an inflammatory disorder characterized by irreversible morphologic
changes
Chronic alcohol abuse is the most common cause in industrialized countries
Young adults with chronic pancreatitis require genetic tests for cystic fibrosis
Diagnosis
The diagnosis of chronic pancreatitis can be challenging since laboratory studies and
imaging procedures may be normal
The classic triad of pancreatic calcifications, steatorrhea, and diabetes strongly suggests
the diagnosis
The most sensitive test for pancreatic function is the secretin stimulation test
Normal amylase and lipase levels do not rule out chronic pancreatitis
Treatment
Directed at controlling pain and alleviating the manifestations of diabetes mellitus,
malabsorption, and steatorrhea
Initial therapy is administration of pancreatic enzymes
Corticosteroids can be used for autoimmune pancreatitis (a type of chronic pancreatitis
characterized by hypergammaglobulinemia, pancreatic enlargement, a positive ANA titer)
for a duration of 4-6 weeks, and repeated imaging used to monitor for improvement
Recent update guidelines: Patients with IgG4 autoimmune pancreatitis have a higher risk
of relapse if not maintained on an immunosuppressant, and should be maintained on
azathioprine
Pancreatic Adenocarcinoma
Clinical Presentation
The initial presentation varies according to tumor location
Tumors in the pancreatic body or tail usually present with pain and weight loss
Those in the head typically present with jaundice, pain, weight loss, and steatorrhea
Diagnosis
Transabdominal Ultrasound is the first line because of its higher sensitivity (>90%) for all
pancreatic tumors; in contrast, triple-phase helical multi-detector row CT abdomen is 100%
sensitive for tumors >2cm, but 77% sensitive for tumors <2cm
All patients require biopsy of a pancreatic mass that can be obtained by endoscopic
ultrasonography with fine-needle aspiration biopsy
Serum tumor markers (CA 19-9) are not used to diagnose pancreatic cancer
Treatment
Surgical resection is appropriate for patients with resectable cancer
For advanced disease treatment is controversial
Survival rate is usually 6 months or less for patients with metastatic disease
Palliation of biliary obstruction should be considered
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Cystic Neoplasms of the Pancreas
Many pancreatic cysts are discovered incidentally when abdominal imaging is obtained for
unrelated indications
Treatment of benign lesions consists of observation and follow-up
Genetics
Adenoma-to-carcinoma sequence
The progression from normal mucosa to adenoma to cancer takes approximately 15 years
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Staging of Colon Cancer
The TNM staging is the preferred staging system for colon cancer
Viral Hepatitis
Hepatitis A Virus
Transmitted via the fecal-oral route, and is more prevalent in low socioeconomic areas
Self-limited illness and rarely leads to fulminant hepatitis
Severity of infection can vary from a mild flu-like illness to fulminant hepatitis
Laboratory findings include marked increase in serum aminotransferases (usually >1,000
U/L), serum total and direct bilirubin, and alkaline phosphatase
Diagnostic test: IgM antibodies to HAV (IgM anti-HAV)
2011 Updated Guidelines on HAV Vaccination indications: travel to endemic countries, HIV
patients, chronic liver disease, exposure in outbreak, post-exposure prophylaxis, close
contact with international adoptees
Management
Hepatitis B e antigen (HBsAg) positive
HBeAg positive
Alanine aminotransferase (ALT) <1x ULN
Every 3 to 6 month ALT - Every 6 to 12 month HBeAg
ALT 1-2x ULN
Every 3 month ALT - Every 6 month HBeAg - Possible biopsy and treat if
over 40
ALT >2x ULN - Hepatitis B virus (HBV) DNA>20,000 IU/mL
Every 1 to 3 month ALT, HBeAg for spontaneous conversion - Possible
biopsy and treatment
HBeAg negative
ALT >2x ULN - HBV DNA>20,000 IU/mL
Treat
ALT 1-2x ULN - HBV DNA 2,000-20,000 IU/mL
Possible biopsy and treatment
ALT <1x ULN - HBV DNA<20,000 IU/mL
Every 3 month ALT x3, followed by every 6 to 12 month ALT if still <1x ULN
Indications for hep B Vaccine: people born in endemic areas, patients with chronically
elevated aminotransferases, HIV, or HCV patients, household, family or sexual partners of
HBV patients, men who have sex with men, patients with multiple sexual partners, prison
inmates, IVDA, dialysis patients, and immunosuppressed patients
The 2008 NIH Guideline for Rx criteria : compensated cirrhotics with HBV DNA >2000,
patients with acute liver failure, decompensated cirrhosis, high HBV DNA in cirrhotics,,
chemotherapy patients, HBeAg-positive patients. Treatment options: Interferon,
lamivudine, Entecavir, tenofovir, telbivudine, etc.
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Hepatitis C
85% develop chronic infection, 20 to 25% of those develop cirrhosis
Most patients with chronic infection are asymptomatic or have only mild nonspecific
symptoms
High-risk groups include IV drug abusers and recipients of blood transfusions prior to
1992
Measurement of anti-HCV is the initial diagnostic study. If positive, test for HCV RNA
Test for HCV in patients with vasculitis, cryoglobulinemia, glomerulonephritis, and
porphyria cutanea tarda
There are six different genotypes for HCV disease: genotypes 1, 2, 3, 4, 5, 6. Differentiating
between types 1a, and 1b is important because of resistance associated with treatment
Treatment should be offered for patients with compensated liver disease: Peginterferon
combined with ribavirin (plus protease inhibitor boceprevir or telaprevir in patients with
genotype 1)
The newest addition for treatment of HepC is sofosbuvir with interferon and ribavirin:
found to be 90% effective in genotypes 1, 4, 5, 6. Sofosbuvir and ribavirin is 70-95%
effective in genotypes 2 and 3.
The Updated guideline on monitoring treatment response is there is no indication for
periodic viral response to treatment with the newest treatments
Hepatitis D
Due to the dependence of HDV on HBV, the diagnosis of hepatitis D cannot be made in
the absence of HBV infection
Presentation as acute hepatitis or an exacerbation of preexisting chronic hepatitis
Prevention of HDV is vaccination against HBV, its helper virus.
No specific treatment exists; although there are limited evidence of foscarnet in treatment
Hepatitis E
Pregnant woman with acute infection, ADD: malnourished, and patients with chronic liver
diseases are at greatest risk for severe hepatitis or fulminant liver failure
Spread is by fecal-oral route, and outbreaks
Vaccines for Hep E prevention have been developed, but not yet commercially available
Prevention is by using healthy eating and hand hygiene habits when traveling to endemic
countries
Treatment is mainly supportive
Autoimmune Hepatitis
An inflammatory condition of the liver of unknown cause
Presents with weakness, fatigue, jaundice, anorexia, and incidental elevations of
aminotransferases discovered on routine laboratory testing
Up to 50% of patients have a concomitant autoimmune disease, such as thyroiditis,
ulcerative colitis, or synovitis
Liver biopsy is required for definitive diagnosis
Treat with prednisone and azathioprine
There is a high risk of relapse. Patients should be tapered off prednisone after repeated LFT
monitoring, and absence of symptoms
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Wilson Disease
Rare autosomal recessive disorder
Initial screening (when suspected) with liver function tests, a CBC, serum ceruloplasmin,
24-hour basal urinary copper, and slit-lamp examination for Kayser-Fleischer rings
Treatment of Wilson disease is directed at reducing the copper overload with the use of
copper chelators (penicillamine)
First-degree relatives should be screened
Hereditary Hemochromatosis
Hereditary hemochromatosis is an autosomal-recessive disorder (HFE Gene)
Initial evaluation involves measuring the transferrin saturation and the ferritin
HFE gene testing is indicated when the fasting serum transferrin >45%
A nondiagnostic HFE genotype does not rule out the diagnosis
Phlebotomy is the treatment of choice to achieve a Ferritin <50 ng/mL
Cirrhosis
Presenting symptoms are related to hepatocyte dysfunction (jaundice, coagulopathy, and
edema) or due to increased portal venous pressure (ascites, bleeding esophageal varices,
hepatic encephalopathy, or hypersplenism)
The main causes of cirrhosis globally are Hepatitis B (30%), Hepatitis C (27%) and alcohol
(20%), non- alcoholic fatty liver disease
Portal Hypertension
Results from a combination of splanchnic vasodilation and increased resistance to blood
flow due to intrahepatic fibrosis
It is the pressure difference between the portal vein and the hepatic veins
Gastroesophageal Varices
Upper endoscopy is indicated for all new patients to screen for varices
Updated Guidelines for surveillance: EGD surveillance frequency is recommended yearly
in patients with decompensated cirrhosis; 1-2 years in patients with small varices, and 2-3
years in with patients with no varices on screening endoscopy
Hepatic venous pressure gradient is the best risk factor for the development of varices,
with values >10mmHg a red flag
Primary prophylaxis with non selective -blockers is indicated in patients with medium or
large varices
In patients with bleeding varices, Endoscopic variceal ligation or Trans-intrahepatic
portosystemic shunts (TIPS) should be considered
Ascites
Paracentesis should be performed for newly discovered ascites with calculation of the
serum-ascites albumin gradient (SAAG)
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Spontaneous Bacterial Peritonitis
Ascitic fluid granulocyte count >250/L confirms SBP
Ascites in a cirrhotic who presents with fever, abdominal pain, altered mental status,
leukocytosis, azotemia
Treatment with antibiotics (3rd generation cephalosporin for at least 5 days) and albumin
infusion if 4-5L of fluids is removed to prevent renal damage
Hepatorenal Syndrome (HRS)
Diuretic therapy, paracentesis without volume expansion, and GI bleeding may precipitate
HRS
Lack of improvement of renal function following fluid challenge with administration of
11.5 L of normal saline suggests HRS
Almost all patients with HRS require liver transplantation
Hepatopulmonary Syndrome (HPS)
Suspected in patients with dyspnea, hypoxemia, and cirrhosis
Patients may exhibit platypnea (increased dyspnea sitting up and decreased dyspnea
lying flat)
HPS is different from portopulmonary hypertension (pulmonary hypertension secondary
to liver disease)
Hepatic Encephalopathy
Reversible decrease in level of consciousness or personality change with liver failure
Requires evaluation for bleeding, SBP (or other infections), and electrolyte disorders
Prevention by using lactulose their mechanism is by converting ammonia to unabsorbable
ammonium, for easy excretion, and they augment easy transit of contents through the gut
Antibiotics such as rifaximin are recommended to reduced the generation of ammonia by
gut bacteria in encephalopathy
Hepatocellular Carcinoma (HCC)
Patients with cirrhosis should have serum AFP measurement and abdominal
ultrasonography
Order imaging (CT or MRI) when serum AFP levels are >20 ng/mL or abdominal
ultrasonography is abnormal
A serum AFP level >500 ng/mL is diagnostic
Surveillance in patients with chronic liver disease with abdominal ultrasound every six
months is recommended for detection of HCC
AASLD Guidelines 2010: HCC diagnosis if nodules larger than 1cm in diameter on
ultrasound has appearance typical of HCC on MRI or CT
OR growth of a <1cm lesion found on ultrasound should be followed every 3 to 6 months,
and if no growth after 2 years, provider should revert to routine screening
Treatment with surgical resection or liver transplantation, sorafenib improves overall
survival in patients with advanced HCC
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ERCP is diagnostic and therapeutic but invasive, MRCP is a non invasive alternative
Treatment in acute cholangitis: Admit to ICU: start on broad-spectrum antibiotics: studies
showed beta-lactam therapy is less toxic and as effective as ampicillin and gentamycin
IV hydration
Mirizzi Syndrome
A condition where the cystic duct obstructs the common bile duct (extrinsic obstruction)
by enlargement of a gallstone in the cystic duct to the point it compresses the common
bile duct
Complications: obstructive jaundice, inflammation and cholecystocholedochal fistula
Diagnosis: Ultrasound or CT scan, and ERCP prior to Surgery
Treatment: cholecystectomy
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10. Schneider G, Siveke JT, Eckel F, Schmid RM. Pancreatic cancer: basic and clinical aspects.
Gastroenterology. 2005;128:1606-25.
11. Whitcomb DC. Clinical practice: Acute pancreatitis. N Engl J Med. 2006;354:2142-50.
12. American College of Gastroenterology. An Evidence-Based Systematic Review on the Management
of Irritable Bowel Syndrome. American College of Gastroenterology Task Force on IBS. Am J
Gastroenterol. 2009;104: Suppl 1.
13. Mayer, EA. Irritable Bowel Syndrome. N Engl J Med. 2008;358:1692-9.
14. Jemal, A, Siegel, R, Ward, E, et al. Cancer Statistics, 2009. CA Cancer J Clin. 2009; 59:225.
15. Screening and Surveillance for the Early Detection of Colorectal Cancer and Adenomatous Polyps,
2008: A Joint Guideline from the American Cancer Society, the US Multi-Society Task Force on
Colorectal Cancer, and the American College of Radiology.
16. Davila RE, Rajan E, Baron TH, et al; Standards of Practice Committee, American Society for
Gastrointestinal Endoscopy. ASGE guideline: colorectal cancer screening and surveillance [erratum
in Gastrointest Endosc. 2006;63:892]. Gastrointest Endosc. 2006;63:546-57.
17. Pratt DS, Kaplan MM. Evaluation of abnormal liver-enzyme results in asymptomatic patients. N Engl
J Med. 2000; 342:1266.
18. Kaplan MM, Gershwin ME. Primary biliary cirrhosis [erratum in N Engl J Med. 2006;354:313]. N Engl J
Med. 2005;353:1261-73.
19. Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006; 354:54.
20. Bacon BR, Gordon SC, Lawitz E, Marcellin P, Vierling JM, Zeuzem S, Poordad F, Goodman ZD, Sings
HL, Boparai N, Burroughs M, Brass CA, Albrecht JK, Esteban R; HCV RESPOND-2 Investigators. N
Engl J Med. 2011 Mar 31;364(13):1207-17.
21. Dominguez M, Rincn D, Abraldes JG, et al. A new scoring system for prognostic stratification of
patients with alcoholic hepatitis. Am J Gastroenterol. 2008; 103:2747.
22. Board Basics 2: An Enhanceent of MKSAP. American College of Physicians.
23. Runyon BA, AASLD Practice Guidelines Committee. Management of adult patients with ascites due
to cirrhosis: an update. Hepatology. 2009; 49:2087.
24. Wadei HM, Mai ML, Ahsan N, Gonwa TA. Hepatorenal syndrome: pathophysiology and management.
Clin J Am Soc Nephrol. 2006; 1:1066.
25. Lewis BS. Obscure GI bleeding in the world of capsule endoscopy, push, and double balloon enter-
oscopies. Gastrointest Endosc. 2007;66: S66-8.
26. Targownik LE, Nabalamba A. Trends in management and outcomes of acute nonvariceal upper
gastrointestinal bleeding: 1993-2003 [erratum in Clin Gastroenterol Hepatol. 2007;5:403]. Clin
Gastroenterol Hepatol. 2006;4:1459-1466.
27. Bardou M, Benhaberou-Brun D, Le Ray I, Barkun AN. Diagnosis and management of nonvari-
ceal upper gastrointestinal bleeding. Nat Rev Gastroenterol Hepatol. 2012 Jan 10;9(2):97-104. doi:
10.1038/nrgastro.2011.260.
28. Longstreth GF, Thompson WG, Chey WD, et al. Functional bowel disorders. Gastroenterology 2006;
130:1480.
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NOTES
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NOTES
C o n t e n t s
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17.1 Physical Examination
Cardiac Sounds
S1 is best heard at the heart apex
Conditions may affect the intensity of S1
S1 sound intensity will decrease in the following conditions:
Prolonged PR interval (1st degree block)
Low ejection fraction (systolic heart failure)
Acute aortic valve regurgitation (MV will close prematurely)
Conditions which affect leaflets mobility of the MV (thickening and calcification)
S1 sound intensity will increase in the following conditions:
If the PR interval shortens
Mitral valve stenosis (with mobile leaflets)
Cardiac hyperdynamic state (fever, anemia, hyperthyroidism)
S1 sound intensity will be variable in the following conditions:
Atrial fibrillation
Atrioventricular (AV) dissociation
The splitting of S2 (best heard at the left lower sternum using the diaphragm of the
stethoscope) normally occurs during inspiration
Conditions affect the intensity of S2 sound
S2 sound intensity will decrease in the following conditions:
Severe aortic stenosis
Pulmonic valve stenosis
2 sound intensity will increase in the following conditions:
S
Uncontrolled hypertension
Cardiac hyperdynamic state
Pulmonary hypertension
Pulmonary embolism (P2 increased)
Conditions affect the splitting of S2 sound
Fixed splitting (the splitting of S2 can be heard during expiration and inspiration and
stays the same) atrial septal defect
Persistent splitting (the splitting does not disappear in expiration but gets wider with
inspiration) pulmonic stenosis, pulmonary embolism, right bundle branch block
(RBBB), left ventricular pacing (biventricular pacemaker or ectopic beat originating
from LV)
Paradoxical splitting (A2 after P2 and happens during expiration); Severe AS,
hypertrophic cardiomyopathy, left bundle branch block (LBBB), right ventricular pacing
(pacemaker or ectopic beat originating in right ventricle)
S3 is best heard by the bell of the stethoscope, at the apex (left ventricular gallop)
or at the lower sternum (right ventricular gallop)
S3 is normal in pregnant and young athletes, otherwise indicates ventricular failure
S4 results from filling of a stiff ventricle by strong atrial contraction
S4 is best heard by the bell at the apex (left ventricular S4) or left lower sternum
(right ventricular S4)
Cardiac Murmurs
In general, the murmur will get louder if the flow across the valve increases
Squatting, for instance, will increase the venous return and the flow so it will intensify all
types of murmurs (except murmur associated with HOCM)
On the other hand, standing and Valsalva decrease the blood return and soften all
murmurs (except HOCH murmur which increases)
Murmurs Associated With Left Heart Lesions
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Murmurs Associated With Right Heart Lesions
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17.3 Coronary Artery Disease (CAD)
>50% obstruction in any epicardial coronary artery and mostly a result of atherosclerosis
If the stenosis is significant it will impair blood flow
Ischemia results when disequilibrium between myocardial oxygen supply and demand
occurs
As a consequence of ischemia, diastolic dysfunction happens followed by systolic
dysfunction, and then the EKG changes will appear and the symptoms of chest pain start
CAD may manifest as chronic stable CAD, acute coronary syndrome, heart failure, sudden
cardiac death, or silent disease (asymptomatic, but have EKG or imaging changes)
Chronic stable CAD is the most common type (50%) of ischemic heart disease
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Coronary revascularization can be by percutaneous coronary intervention for coronary
bypass surgery (CABG)
PCI has the limitation of restenosis; however, using drug-eluting stents (DES) decreases
neointimal hyperplasia and significantly reduces the restenosis to <15%. Antiplatelet
therapy with clopidogrel should be given for at least 12 months if DES was used, and at
least for 1 month in patients who received bare metal stents (BMS). Aspirin should be
given indefinitely decrease the rate of stent thrombosis
CABG is indicated in patients with left main artery disease and/or 3-vessel disease
2-vessel disease and 1 of the vessels is the proximal LAD (especially in patients with
EF <50% or demonstrable ischemia on nuclear or echo stress test) if the patient had
recovered sudden cardiac arrest or sustained ventricular tachyarrhythmia and the
coronary angiogram showed significant disease in any coronary artery
Generally diabetics and patient with reduced EF do better with surgical
revascularization in comparison to PCI/stenting
Antiplatelet should be held appropriately before elective CABG (clopidogrel 5 days,
tirofiban and eptifibatide about 6 hours, abciximab 24 to 48 hours and prophylactic
platelet transfusion)
Arterial grafts have better patency rate than venous graft
CABG associated with cognitive decline and neurological defects with no significant
difference between on-pump and off-pump (no aortic cannulation) approach
Refractory angina
Enhanced external counterpulsation is a treatment option for refractory angina
Transmyocardial laser revascularization; either percutaneous or through thoracotomy.
Its use is controversialit may reduce angina symptoms via unknown mechanism
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Early risk stratification: Factors predicting mortality at presentation include:
Advanced age, heart failure at presentation, tachycardia, cardiogenic shock,
anterior MI, DM, renal failure and calculation of TIMI risk score for STEMI provides
precise information about the rate of in-hospital mortality
STEMI complications could be mechanical complication, usually after 48-hours and
include: Acute MR (papillary muscle rupture), VSD, free ventricular wall rupture
(risk: advanced age, 1st MI, NSAIDs, and steroid), pericarditis, and left ventricular
aneurysm
Conditions with normal coronaries and STEMI presentation include: Coronary
spasm, hypercoagulability, Takotsubo syndrome (stress induced cardiomyopathy),
cocaine use, collagen vascular disease, embolism, myocarditis, microvascular
disease
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Heart failure patients should have serial assessments of their functional status by NYHA
classification, 6-minute walking test, or cardiopulmonary exercise test
Volume status should be assessed serially by physical examination, patients weight, or
ultrasound of the inferior vena cava to assess its size. BNP can be measured if the volume
status is difficult to evaluate
Serial assessments of electrolytes, kidney function, and liver enzymes
Serial assessment of heart failure exacerbating factors
Reassessment of the systolic function by echocardiography should be performed only if
there is change in the clinical condition of the patient
Assessment of the patients prognosis will help in the management of heart failure and
discussing the different treatment options with the patient. There are several models that
can be used (ex.: Seattle Heart Failure Model [SHFM] is a calculator which estimates survival
at baseline, 1, 2, and 5 years in patients with systolic heart failure)
Specific Cardiomyopathies
Takotsubo cardiomyopathy (stress induced cardiomyopathy)
Is a transient acute cardiac systolic dysfunction with a similar presentation to
ST-segment elevation myocardial infarction (STEMI)
TTS usually affects elderly women and is characterized by a new cardiac motion
abnormality that commonly involves the apex of the left ventricle in the absence of
significant obstructive coronary artery disease
Acute, severe emotional or physical stress is believed to be the main precipitating
factor of TTS
Usually it is a benign condition and the systolic function returns to normal in few days
to few weeks
Acute myocarditis
Inflammation of the myocardium accompanied by myocellular necrosis
Can be asymptomatic or may be fulminantresults in severe heart failure symptoms
and cardiogenic shock
Usually the cardiac enzymes are elevated and the echocardiogram may show global
dysfunction and less frequently, regional motion abnormality
The treatment mainly is supportive
Tachycardia-mediated cardiomyopathy
Reversible left ventricular dysfunction usually results due to supraventricular
tachyarrhythmia. It has also been reported as a result of ventricular tachyarrhythmia
The myocardial high energy stores get depleted which will result in cellular and
structural changes
Treatment should be directed toward rate control
Cardiomyopathy is reversible, in weeks to months after heart rate control, in most
cases
Arrhythmogenic right ventricular dysplasia
An important cause of sudden death
Results from fibrofatty infiltration of the right ventricle
Right ventricle usually enlarged and has reduced systolic function while the left
ventricular function is preserved
ECG may show epsilon waves (positive deflection just after the QRS)
Usually diagnosed by cardiac MRI
Giant cell myocarditis
It causes congestive heart failure (biventricular enlargement) and frequently associated
with refractory ventricular arrhythmia
Usually presents as cardiogenic shock
A fatal form of myocarditis
Affects young adults
Heart transplant is the treatment of choice; however, it may recur after cardiac
transplantation
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17.5 Myocardial Disease
Restrictive Cardiomyopathy
A non-dilated myocardial disease characterized by diastolic dysfunction with an increase in
filling pressureresults in pulmonary edema
Majority of the cases are idiopathic; however, amyloidosis is the most common identified
cause
Sarcoidosis and hemochromatosis are also important causes of restrictive cardiomyopathy.
Cardiac MRI is the diagnostic modality of choice to assist myocardial involvement
On examination: JVD, Kussmaul sign, ascites, and hepatomegaly
Echocardiographic findings that suggest amyloidosis include thickened valves, ventricular
hypertrophy (however, ECG will reveal low voltage), and pericardial effusion
Treatment
Diuretics are used to treat the heart failure symptoms
b-blockers and non-dihydropyridines calcium channel blockers improve the relaxation
of the myocardium
Bradyarrhythmias and tachyarrhythmias should be avoided. A pacemaker is helpful in
symptomatic patients with bradycardia. Cardioverting patients with atrial fibrillation will
help improve the filling of the ventricle
Treatment of underlying cause will help in improving the diastolic function
In patients with hemochromatosis, frequent phlebotomy and chelating agents may
be helpful
In patients with sarcoidosis, the use of steroid therapy can improve survival
Stem cell transplant and cardiac transplantation in specific type of amyloidosis is
an option in patients with severe heart failure
Hypertrophic Cardiomyopathy
Genetic disordersusually inherited as autosomal dominant. Not all patients have family
history because of variable penetrance
Frequently affects African American males
Characterized by inappropriate left ventricular hypertrophic changes, which may affect any
region of the left ventricle, but can be diffuse
Interventricular septum is frequently the region that gets affected and may result in left
ventricular outflow obstruction with significant hemodynamic changes
The basal posterior segment usually spared
Cardiac Tumors
Most tumors of the heart have metastatic involvement
Primary heart tumors are very rare
Majority of primary cardiac tumors are benign
Usually diagnosed incidentally by echocardiogram
TEE or cardiac MRI/ CT is sometimes needed for better, more detailed images
Clinical manifestation may include constitutional symptoms, systemic embolization (stroke,
TIA), mechanical obstruction of the heart valve or outflow tract, valve regurgitation,
arrhythmia, and heart failure symptoms
Atrial myxoma is the most common type of primary tumors
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Originate in the left atrium and widely differ in size
Can be part of the Carney complex (an autosomal dominant disorder which includes
cardiac and extracardiac neoplasms)
Myxoma should be resected to decrease the risk of systemic embolization and risk of
sudden death
17.6 Arrhythmias
Antiarrhythmic Agents
Class IA
Causes QT prolongation
Chronic use of procainamide is associated with lupus
Disopyramide is effective in treating parasympathetic mediated atrial fibrillation
Class IB
They are not used in atrial fibrillation management because of minimal effect on the
atrium
Class IC
The QRS duration should be monitored since they may cause widening of QRS
Should not be used in patients with structural heart disease or post-MI ( mortality)
Not used alone in managing atrial fibrillation. Should be used with b-blocker or calcium
channel blocker to slow the conduction through the AV node since class IC may result
in conversion of atrial fibrillation to atrial flutter with 1:1 conduction
Class III
Sotalol: A mixture of isomers (l-sotalol and d-sotalol)
Contraindicated in decompensated heart failure and can cause QT prolongation
Dofetilide: Contraindicated if QT interval is prolonged at baseline. ECG should be
obtained at baseline and after each dose to follow the QT interval
If QTc exceeded 500 ms it should be stopped
Dofetilide is not contraindicated in heart failure
Atrioventricular Block
There is abnormal conduction of the electrical impulse through the AV node and
conduction system because of fibrosis and degeneration of the electrical conduction
system
Coronary artery disease and medications are also important causes to be excluded
AV Dissociation
The atrial and ventricle are beating independently. Usually the ventricular rate is the same or
higher than the atrial rate
Tachycardia
ECG should be obtained during the episode of palpitation.Holter monitor often needed to
record the tachycardia
Management: 3 steps are important to follow for appropriate management
Identify if the patient is stable or not; if hemodynamic instability is present,
cardioversion is the appropriate treatment
Check if the rhythm is regular (SVT or ventricular tachycardia) or irregular (atrial
fibrillation)
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Calculate QRS duration: If QRS >120 ms (wide complex): Ventricular tachycardia (VT),
SVT with aberrant conduction or ventricular pre-excitation (accessory pathway). VT
should be differentiated from the other forms of wide complex tachycardia, otherwise
should be treated as VT. If QRS <120 (narrow complex): SVT. Adenosine, calcium
channel blocker or b-blocker is the treatment for stable patients
Atrial Fibrillation
Most common arrhythmia in clinical practice
It is either paroxysmal, persistent, or permanent
Clinical presentation: It can be asymptomatic but common symptoms at presentation
include palpitation, dyspnea, and dizziness. Thromboembolism may result in stroke,
acute limb ischemia, and renal embolism (acute kidney injury, decrease urine output, and
eosinophils in urine)
If the heart rate is not controlled it may result in tachycardia induced cardiomyopathies
Atrial fibrillation commonly associated with hypertension, valvular heart disease, coronary
heart disease, and cardiomyopathies
Atrial fibrillation is a common complication of cardiac surgery
Atrial fibrillation patients should be evaluated for reversible causes which include; thyroid
disorder, coronary artery disease, alcohol use, myocarditis, pericarditis, and pulmonary
embolism
Acute Management
If the patient is not stable or developed cardiac ischemia secondary to uncontrolled atrial
fibrillation, cardioversion should be performed
If the patient is stable the rate can be controlled using b-blocker or calcium channel
blocker. Digoxin can be added particularly in elderly and those with heart failure
AV nodal blocking agents are contraindicated in patients with preexisted atrial fibrillation
because it may increase the conduction through the accessory pathway and can lead to
ventricular fibrillation
If the patient has atrial fibrillation for less than 48 hours, synchronized cardioversion can
be performed without anticoagulation
If the patient has atrial fibrillation for longer than 48 hours or if the duration is unknown,
the patient can either be anticoagulated with intravenous heparin and can undergo
synchronized cardioversion if intra-cardiac clot was excluded by transesophageal
echocardiogram, or the anticoagulation can be continued for 3 weeks and the INR should
be between 2 to 3, then synchronized cardioversion can be arranged
All patients who underwent either mechanical or pharmacological cardioversion should
be anticoagulated for 4 weeks (atrial mechanical dissociation risk of clot formation post
cardioversion)
In patients who have pacemakers and intracardiac defibrillators cardioversion is safe but
pads should be away from them
Long-term Management
Rate control
Commonly using b-blocker and nondihydropyridine calcium channel blocker
The heart rate at rest should be less than 80. The heart rate when exercising should
be less than 115 beats/minute
Controlling the heart rate will prevent tachycardia induced cardiomyopathy
Atrial Flutter
Clinical Presentation and Evaluation
Common symptoms include palpitations, fatigue, dizziness, and mild dyspnea
Atrial flutter can be as a result of cardiac disease (post cardiac surgery, congenital
cardiac anomaly, cardiac ischemia, sick sinus disease, cardiomyopathies, HTN, valvular
disease) or associated with systemic disease (pulmonary embolism, chronic obstructive
pulmonary disease, diabetes, hyperthyroidism, obesity) or may result from substance
abuse (commonly alcohol and cocaine)
ECG is important to confirm the diagnosis and determining the type
Echocardiogram can help assess the systolic function, size of the atria, valve disease
(rheumatic mitral valve disease), and presence of intracardiac thrombus
Acute Management
If the patient is hemodynamically unstable, synchronized electrical cardioversion is the
appropriate treatment
Pharmacologic cardioversion (Class III antiarrhythmics: Ibutilide is the most effective) can
be tried in stable patients
If atrial flutter is more than 48 hours or of unknown duration, TEE or anticoagulation for 3
weeks should be performed before cardioversion (similar to atrial fibrillation)
Rate control can commonly be achieved using b-blockers and nondihydropyridine
calcium channel blockers
Chronic Management
If atrial flutter is recurrent, radiofrequency ablation is the treatment of choice with high
success rate
Anticoagulation therapy post ablation treatment is needed in patient with high CHADS2
score
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Supraventricular Tachycardia
AV nodal reentrant tachycardia (AVNRT) is the most common followed by AV reentrant
tachycardia (AVRT). Atrial tachycardia is the least common
2 types of AVNRT:
Typical: The conduction to ventricle is through the slow pathway and retrograde
conduction through the fast pathway (short RP)
Atypical: The conduction to ventricle is through the fast pathway and retrograde
conduction through the slow pathway (long RP)
AVRT: There is accessory pathway conducts from the atrium to ventricle. If the accessory
pathway conducts anterograde, the ventricular preexcitation can be seen as delta waves.
However, if the accessory tracts only conducts retrograde, it will not be identified by ECG
and will be called concealed
Clinical Presentation and Evaluation
Episodes of palpitation start and terminate abruptly. Symptoms can differ widely from
mild dyspnea to syncope
Frequently, event-monitor is needed to capture the arrhythmia
Underlying exacerbation condition should be excluded including anemia, hyperthyroidism,
and sepsis
Patient with WPW syndrome who had SVT should have the accessory pathway ablated
since they are at risk of sudden death (first-line therapy)
Acute Management
Carotid massage and other vagus-nerve stimulating maneuvers should be tried first in
stable patients
Adenosine (IV bolus, contraindicated in severe asthma) usually effective in terminating
AVNRT/AVRT
Other drugs include intravenous calcium channel blocker, intravenous b-blockers, and
amiodarone
Synchronized cardioversion should be the treatment in patients who are
hemodynamically unstable
Atrial tachycardia is usually resistant to cardioversion and adenosine (enhanced atomicity
mechanism)
Long-term Management
If SVT episodes are infrequent, observation and avoiding exacerbating factors would be
appropriate
b-blockers and calcium channel blockers can be used to decrease the recurrence.
However, they should be used cautiously in patients with WPW syndrome
Ablation has a high success rate (AVNRT/AVRT more success than atrial tachycardia)
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Lange-Nielsen syndrome (autosomal-recessive pattern of inheritance and associated with
congenital deafness)
b-blockers are first-line therapy and have been shown to decrease overall mortality
ICD is indicated in patients with recurrent symptoms
Short QT Syndrome
QTc interval <300 ms and usually associated with peaked T waves
Inherited (ryanodine receptor mutation)
Polymorphic V-tach usually happens during emotional stress or activity
ICD placement may be considered to prevent V-tach and SCD
It is associated with increased sudden death
Brugada Syndrome
Brugada pattern is characterized by J-point elevation in V1-V3 and right bundle branch
block morphology
These changes can be intermittent and can be precipitated by several factors including
fever and electrolytes disturbances
If the Brugada pattern ECG changes associated with syncope, it is called Brugada
syndrome and has increased risk of sudden death
Acute Pericarditis
Idiopathic (majority)other common causes include: Infectious, uremic, malignancy,
radiation, collagen vascular disease, post-MI, and medications
Past medical history should be reviewed for clues to specific etiologies
Constrictive Pericarditis
Common causes include: Post-pericarditis, post-radiation, post-cardiac surgery, and
tuberculosis
Physical examination: Jugular venous distension, hepatomegaly, ascites, and peripheral
edema. Early diastolic knock and Kussmaul sign can also be found. Constrictive
pericarditis does not usually cause pulsus paradoxus
Calcification of the pericardium (in up to 50% of patients) can be best visualized on the
lateral projection. CT scan and cardiac MRI will show thickening in most cases
The diagnosis can be confirmed by cardiac catheterization which reveals elevation and
equalization of diastolic pressures in each of the cardiac chambers
Unlike cardiac tamponade, there is a prominent descent of the right atrial pressure tracing
Treatment of constrictive pericarditis requires surgical removal of the rigid pericardium
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Echocardiography is indicated in:
Symptomatic patients
Patients with heart murmur (continuous, at least moderate-intensity systolic murmur, or
diastolic murmur)
BNP has a prognostic value and helps with understanding the hemodynamic significance of
the valve lesion
Aortic Regurgitation
May result from valve disease or root pathology
Can be acute (medical emergency) or chronic
Acute Aortic Regurgitation
Common causes include: Aortic dissection (type A), infective endocarditis, chest trauma,
and malfunction of prosthetic valve
The LV is not dilated so acute change in volume load will result in rapid increase in the
end-diastolic pressure and pulmonary edema will develop
Typically patients present with sudden dyspnea which may rapidly progress to heart
failure, chest pain ( myocardial perfusion pressure), and cardiogenic shock
On examination S1 will be soft and S3 may be present. Short diastolic murmur can be
heard; however, if the patient has tachycardia it can be inaudible
Acute severe AR has high rates of morbidity and mortality
Transthoracic echocardiography should be the diagnostic modality of choice if acute AR
is suspected
Transesophageal echocardiogram, CT, or MRI should be performed if dissection is
suspected as mechanism of the acute AR
Intra-aortic balloon pump is contraindicated in AR
Surgical intervention is usually the treatment
Chronic Aortic Regurgitation
Causes gradual increase in ventricular volume which results in ventricular enlargement
and hypertrophy
In early stages the ejection fraction (EF) is normal or even increased and patients may
remain asymptomatic during this time. However, as AR progresses the left ventricle
cannot accommodate the increase in volume and diastolic pressure begins to rise,
resulting in symptoms. Later, LV will enlarge and the EF starts falling to normal and then
subnormal levels
Usually patients presents with palpitations, shortness of breath, and chest pain
On physical examination, the point of maximal impulse usually diffuses and is
hyperdynamic. Pulses are bounding. S3gallop (LV dysfunction), high-pitched diastolic
murmur (duration of murmur correlates with the severity) and Austin Flint murmur
(middiastolic low-pitched murmur results from early closure of the mitral valve by aortic
regurgitant jet) may be audible on auscultation
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Bicuspid aortic valve, infective endocarditis, aortic valve prolapse, connective tissue
disorders, rheumatic fever, aortitis (ankylosing spondylitis, Behet disease, Giant cell
arteritis, Takayasu arteritis), rheumatoid arthritis (by forming granulomatous nodules
within the valve leaflets) are possible causes of AR
Aorticvalve replacement is recommended for patients:
With chronic severe AR who are symptomatic or resting EF of <55%
Who are asymptomatic, with LV end-systolic dimension >55 mm
Mitral Stenosis
Causes obstruction of the left ventricular inflow at the level of mitral valve
Rheumatic fever is the most common cause of mitral stenosis (commissural adhesion)
The clinical presentation of rheumatic mitral stenosis is usually between the 4th and 5th
decades but can present earlier if the disease is severe
Other etiologies include congenital mitral stenosis, carcinoid disease, systemic lupus
erythematosus,rheumatoid arthritis, severe mitral annular calcification, infective
endocarditis with large vegetation, left atrial myxoma, mechanical valve thrombus, or cor
triatriatum (congenital anomaly of the atrium)
Mitral valve is usually above 4 cm2. If the area started to decrease by valve pathology the
pressure gradient across the valve will start to rise to maintain the flow. If the pressure
gradient is more than 10 mmHg and the valve area less than 1 cm2, the stenosis is
considered severe
Clinical presentation includes dyspnea on minimal exertion or at rest. Hemoptysis (because
of bronchial venule rupture), hoarseness (the enlarged atrium will compress the recurrent
laryngeal nerve), frequent coughing (bronchial irritation by enlarge atrium), and atrial
fibrillation (risk of systemic embolization)
Physical examination will reveal jugular vein distension.Right ventricular lift can be felt in
patients with pulmonary hypertension. Opening snap (will be closer to S2 in severe MS)
followed by low pitch, and diastolic murmur, which is best heard at the apex (duration
correlates with the severity). The murmur gets louder near the end of diastole. The murmur
gets softer with Valsalva and louder with exercise. Diastolic murmur, because of pulmonary
regurgitation, can also be heard
Management
Rate control is very important in patients, especially with atrial fibrillation to prolong the
filling time of the ventricle
Percutaneous balloon valvotomy is indicated (if the valve morphology is favorable
for balloon valvotomy, otherwise surgical commissurotomy or MVR) in symptomatic
patients (mitral area less <1.5 cm2), asymptomatic with valve area 1.5 and have resting
pulmonary hypertension (PASP >50 mmHg), or exercise induced pulmonary HTN
(PASP >60 mmHg)
Mitral Regurgitation
Chronic MR
Common causes includemitral valve prolapse (MVP), rheumaticcardiac disease, infective
endocarditis, mitral annular calcification (mainly in elderly), cardiomyopathy (usually
because of dilatation of the annulus) and cardiac ischemia (causes dysfunctional papillary
muscle or tethering of the mitral leaflet)
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Mitral Valve Prolapse
MVP is the most common valvular pathology (prevalence of 2%)
The distribution is about the same in both genders
Most patients are asymptomatic but some patients may present with atypical chest
pain and symptoms related to autonomic dysfunction (palpitation, anxiety, orthostasis,
presyncope, syncope)
Usually it is a benign condition but infrequently complications may happen which include
mitral regurgitation (which can progress to sever form in spite of medical therapy),
endocarditis, arrhythmia and sudden cardiac death
The majority of patients with Marfan syndrome have MVP
On examination, midsystolic click can be heard (systolic buckling of the redundant mitral
leaflet) followed by short systolic murmur
MVP is diagnosed by transthoracic echocardiogram (shows thickening of the mitral leaflet
and mitral leaflet prolapsing into the left atrium more than 2 mm)
Endocarditis
Should be suspected in patients with fever and new heart murmur
High risk patients include those with underlying valve disease, prosthetic valves, history of
infective endocarditis (IE), and immunosuppressed patients
Mechanical and bioprosthetic valves have the same long-term risk of infective endocarditis;
however, immediately after valve implantation the mechanical valve has higher risk
Endocarditis commonly leads to CNS embolization. The risk of embolization varies
according to valve and organism. Mitral valve infection and Staphylococcusspecies
infection are the most important determinants of embolization
Staph Aureus and Streptococcus species are common microorganisms to cause infective
endocarditis
Blood cultures are usually positive for microorganisms; however, culture-negative
endocarditis may result from partial treatment with antibiotics. Some organisms may result
in negative blood cultures and include Haemophilus,Actinobacillosiscardiobacterium
hominis, Eikenella corrodens, and Kingella
Prosthetic Valves
There are 2 types of prosthetic valves: Bioprosthetic and mechanical valves
Bioprosthetic
Less durable than mechanical valves because of calcification and degeneration
All patients should be on low-dose aspirin
Warfarin is only indicated for mitral bioprosthetic valve in the first 3 months post surgery,
otherwise no anticoagulation is needed for bioprosthetic valves
Mechanical Valve
All patients should be anticoagulated with warfarin and should be on low-dose aspirin.
The INR target depends on the location of the valve and the patient risk factors
Mechanical aortic valve should have INR of 2 to 3 unless they are at higher risk
for thrombus formation (atrial fibrillation, depressed systolic function, history of
thromboembolism, or hypercoagulable state) the INR should be 2.5 to 3.5
For mechanical mitral valve (low-flow valve with large area) the INR should be 2.5 to 3.5
Prosthetic valve complication includes: Primary valve dysfunction, valve thrombosis,
endocarditis, thromboembolism, and hemorrhage
Transesophageal echocardiogram is the test of choice to evaluate the prosthetic valve
Surgery is the treatment of choice in patients with valve thrombus who present with
heart failure
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Table 17.10: Conginital Cardiac Defects
Atrial Septal Most common CHD in adults 2nd to bicuspid aortic valve
Defect Left to right shunt
Lead to right ventricle volume overload and pulmonary hypertension
Fixed wide splitting of S2, pulmonic systolic murmur usually present due to increased
flow across the pulmonary circulation, and prominent right ventricular impulse may
present
ECG: Shows incomplete right bundle branch block with right axis deviation (in secun-
dum ASD) and left axis deviation in primum ASD
Transthoracic echocardiography is the test of choicereveals right atrial dilation, elevat-
ed pulmonary pressure, and can detect the septum defect
Secundum, primum, and sinus venosus ASDs should be repaired if symptomatic, or evi-
dence of increased pulmonary flow in comparison to systemic flow (Qp:Qs 1.5:1)
Ventricular The most common type of VSD is perimembranoustype
Septal Small VSD are usually asymptomatic but may produce loud systolic murmur. It closes
Defect (VSD)
spontaneously and if persistent, it does not need closure
Echocardiogram can identify the size, location, and cardiac chamber changes associated
with the defect
Patent Frequently results because of maternal rubella infection
Ductus On physical examination, machinery-type continuous murmur (Gibson murmur) can be
Arteriosus heard and pulse pressure is widened
(PDA)
Color Doppler echocardiography is the test of choice to detect the PDA flow
If small and asymptomatic with no history of infective of endocarditis there is no treat-
ment, only observation
If moderate size with left ventricular overload, percutaneous closure of PDA is recom-
mended if there is no pulmonary hypertension. Otherwise, it is contraindicated to close it
Aortic Most commonly involves the descending aortic segment distal to left subclavian artery
Coarctation origin
More commonly affects males
Frequently associated bicuspid aortic valve. May also be associated with Turner syndrome
and intracranial aneurysm
Chest X-ray may show figure 3 sign (result from pre-stenotic and post-stenotic dilata-
tion of the aorta). Notching of the ribs (from collaterals) can be identified by X-ray
Echocardiogram with supra-sternal view can identify coarctation. Pressure gradient
across the narrowing should be measured
MRA and spiral CT is recommended to assess the entire aorta
Treatment: Surgical correction of the segment is indicated in patients with high-pressure
gradient across the coarctation (>20 mmHg)
Pulmonary Associated with Noonan syndrome
Stenosis Echocardiography is the test of choice to evaluate the right ventricle and Doppler evalu-
ation of the pulmonic valve will determine the severity of the stenosis
Balloon valvuloplasty is the treatment of choice
Tetralogy of Overriding aorta, VSD pulmonary outflow stenosis, and right ventricular hypertrophy.
Fallot (TOF) ASD may present in small portion (pentalogy of Fallot)
Cyanotic and cannot live without correction
Surgical repair before the age of 1 year
Sudden death, secondary to ventricular arrhythmias, is considered the most common
cause of late death post repair
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Dissection
Enlargement >1 cm per year
If the size of the ascending and descending aorta is more than 5.5 cm and 6.5 cm
Marfan Syndrome
Autosomal dominant disorder resulting from fibrillin-1 gene mutation
Increased risk of aortic root dilation and dissection
Serial semiannual echocardiograms are recommended to follow the size of the aortic root
Medical management includes b-blocker and losartan (decreases the rate of aneurysmal
dilation)
Aortic root repair should be performed in Marfan patients if:
The aortic root size is >5 cm (>4.5 cm in women planning to get pregnant)
If the rate of expansion is >1 cm per year
Patients with Marfan should not be involved in heavy activity
Takayasu Arteritis
An infrequent large artery vasculitis (affects the aorta and the major branches) which
mainly affects women at young ages
The vascular clinical presentation depends on the arterial segments involved. Carotids
and vertebrobasilar involvement will result in neurological symptoms (headache, syncope,
TIA, stroke). If renal arteries are involved it will result in uncontrolled hypertension, arm
claudication, and inter-arm pressure difference as a result of subclavian artery involvement
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17.12 Pregnancy and Cardiovascular Disease
Hemodynamic Changes in Pregnancy
plasma volume: Peak by the 2nd trimester (50% of baseline)
Heart rate increase by 20% above baseline, cardiac output (30 to 50%), vascular
resistance slight in blood pressure
venous pressure in lower extremities (80% have pedal edema)
Common normal cardiac symptoms during healthy pregnancy: exercise capacity,
dyspnea, palpitation (APC/VPC)
Physical examination of a healthy pregnancy may look like a patient with heart disease
( S1 and S2 intensity, + S3, + JV distention, systolic murmur [80% of pregnant]), and leg
edema
Normal ECG finding can be found in a healthy pregnancy (axis change, small Q-wave in
lead III, R/S wave ratio in V1-2, sinus tachycardia, arrhythmia)
Always vaginal delivery preferable except if contraindicated
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Prosthetic valve management
Bioprosthetic valve does not need anticoagulation
Mechanical valves need anticoagulation and this is associated with increased risk of
bleeding (maternal and fetal bleeding complication)
Coumadin crosses the placentashould be avoided in the 1st trimester (warfarin
embryopathy, incidence 4 to 10%). Heparin can be given during the 1st trimester and
after 36 weeks. Heparin infusions should be discontinued 4 hours before cesarean
sections
Arrhythmia
Premature atrial contractions and premature ventricular contraction are the most
common
There is an increase risk of SVT
Drugs should be avoided in the 1st trimester
Patients who have life-threatening ventricular arrhythmia should be managed by
implantable defibrillator
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NOTES
R2 2/6/15
154INTERNAL MEDICINE IN-REVIEW l INREVIEWIM.COM
2017 Educational Testing and Assessment Systems (ETAS). All Rights Reserved. This document contains proprietary information, images, and marks of ETAS.
No reproduction or use of any portion of the contents of these materials may be made without the express written consent of ETAS. Licensed to gee.icloud@icloud.com.