Immune

Systemic Lupus Erythematosus

SLE is an autoimmune disease that can
affect any body system.

Prevalence and Incidence
1 per 2500 persons
Occurs 10x more frequently in women than
men
3x more frequently in African Americans
than Caucasians


Systemic Lupus Erythematosus
Pathophysiology
Disturbed immune regulation that causes
an exaggerated production of
autoantibodies
B & T cells contribute to the immune
response
Brought on by:
Genetic factors
Hormonal factors
Environmental factors
Medications
Systemic Lupus Erythematosus
Clinical Manifestations
Musculoskeletal
Arthralgias
Arthritis
Skin
Subacute cutaneous lupus
erythematosus
Discoid lupus erythematosus
Acute cutaneous lesion (50% of
patients with SLE)



Systemic Lupus Erythematosus
Clinical Manifestations (cont.)
Cardiac
Pericarditis
Early artherosclerosis
Renal
Damage as evidenced by elevated
creatinine levels
Central nervous system
Changes in behavior patterns
Changes in cognitive ability
Systemic Lupus Erythematosus
Assessment
Complete history and physical
Focused assessment
Skin assessment- Rashes
Heart sounds- Auscultation for pericardial
friction rub
Lung sounds- Pleural effusions/infiltrations
Joints- swelling, tenderness, warmth, pain,
edema
Neurologic- behavior changes
Systemic Lupus Erythematosus
Diagnostic
Blood test
Moderate to severe anemia
Thrombocytopenia
Leukocytosis or leukopenia
Positive ANA
Systemic Lupus Erythematosus
Pharmacologic Management
Corticosteriods
Antimalarial medications
NSAIDs
Immunosuppressive agents
B cell depleting therapies

What is it?

More commonly called scleroderma

A progressive autoimmune disease

Abnormal growth of connective tissue results
in hard, thick skin and, in more severe cases,
life-threatening damage to other organs

Systemic Sclerosis
Etiology & Incidence

Scleroderma
Affects women 3x more than men
Usually occurs between 30 and 50
Genetics may play a role
Environmental triggers
Pathophysiology

Production of too much collagen for
no apparent reason

Forms connective tissue that builds up
around the cells of the skin and
blood vessels
Scleroderma
Manifestations and Complications.
Thickening, tightening skin
Problems with lungs, heart, and GI tract
Scleroderma
Scleroderma
Two main types
Localized
Gradual onset
Affects the skin in limited
areas

Systemic
More rapid onset
More widespread skin
changes

Localized
Morphea
Reddish patches of skin thicken into
firm, oval shape
Most common on torso but can
appear on arms, legs, or forehead
Generally fades in 3 to 5 years
Scleroderma
Linear
Bands or streaks of thick or abnormally
colored skin running down arms or legs
Occasionally appears down forehead
(en coupe de sabre)
Usually occurs before age 10
Rarely progresses to systemic
scleroderma
Scleroderma
Limited scleroderma also known as CREST
syndrome

Diffuse disease-Affects internal organs as
well as skin

Sine scleroderma- (Some recognize this as
a 3
rd
type of systemic Scleroderma) Affects
internal organs but not skin


Systemic Scleroderma
Diffuse scleroderma
Skin thickening over much of the body
Progresses rapidly for 3 to 5 years
If untreated, can be fatal within several
years of onset
A period of rapid progression may be
followed by a period of stability with little
or no further organ damage

Scleroderma
CREST syndrome
Calcinosis calcium deposits in the
skin
Raynaudss phenomenom
Esophageal hardening & dysfunction
Sclerodactyly tightening of the skin
on the hands and toes
Telangiectasia red lesions on the
hands, forearms, and face caused
by the dilation of blood vessels


What is it?
Vascular vasospasms
What causes it?
Exposure to cold
Emotional distress

Raynauds phenomenon
Manifestations
Skin appears white in response to spasm
May then turn blue (lack of 0
2
-rich blood
Then turns red as blood returns
May also be tingling, swelling, and painful
throbbing
Tissue damage may lead to ulcers, scars, or
gangrene requiring amputation

Raynauds phenomenon
Most common initial symptom
Occurs in 95% of patients with
scleroderma
May experience Raynauds for years
before other symptoms develop
Raynauds phenomenon
Raynauds phenomenon
Raynauds phenomenon
Sclerodactyly
Tightening and thickening of the skin
on the fingers and toes
Skin may swell, appear shiny, and
feel tight and itchy
Edema of the fingers, hands, arms,
feet, or face is an early symptom
Sclerodactyly
Telangiectasia
Signs/Symptoms
Microvascular abnormalities (Vascular injury
that reduces blood flow through the small
arteries, arterioles, and capillaries of the skin, and
in some patients, other organs)
Kidney failure
Pulmonary hypertension
Cardiac dysfunction
Systemic Scleroderma
Musculoskeletal
Joint pain with occasional joint swelling is
common
As the skin becomes stiffer, the joints will also
stiffen, resulting in flexion contractures and
limited mobility
Systemic Scleroderma
GI
reflux, dysphagia, abdominal
bloating, and constipation
alternating with diarrhea
Lower portions of esophagus
and sometimes stomach and
intestine, normal smooth muscle is
replaced by fibrotic tissue, and normal
peristalsis is not possible; eating,
absorbing nutrients, and elimination
may all become difficult


Systemic Scleroderma
Renal
Patients may initially have
proteinuria and a sudden rise in
blood pressure
Reduced kidney function can
result in failure
Systemic Scleroderma
Pulmonary
Early phases, usually
asymptomatic
With progression
Dry cough and dyspnea with
exertion
About 10 to 15% develop severe
lung disease
Systemic Scleroderma
Pulmonary (cont.)
Pulmonary fibrosis
Pulmonary hypertension (can result in
symptoms similar to CHF)
May have both
Pleurisy and pleural
effusions are common
Systemic Scleroderma
Cardiac
Problems related to scarring and
inflammation of heart tissue
Cardiac muscle fibers replaced by fibrotic
tissue, which affects the conduction
system and can lead to arrhythmias,
cardiomyopathy, myocarditis and
pericarditis
Systemic Scleroderma
Diagnostics
Diagnosis is difficult
No single definitive test
Clinical history and physical findings
Systemic Scleroderma
Diagnostics (cont.)
Nail fold capillary test.
Uses a microscope to examine
the skin beneath a patients
fingernails to look for normal
capillary function
Skin biopsy
Systemic Scleroderma
Treatment
No CURE!
Symptomatic
Raynauds phenomenon
Calcium channel blockers
Nifedipine (Procardia, Adalat)
alpha-blockers
Doxazosin mesylate (Cardura)
Systemic Scleroderma
Treatment (cont.)
ACE inhibitors
Captopril (Capoten)
Benazepril HCl (Lotensin)
Lisinopril (Prinivil, Zestril)
Angiotensin II receptor blocker (losartan
potassium (Cozaar) can reduce the number of
episodes of Raynauds
Nitroglycerin topical ointment (Nitrol, Nitro-bid)
can be applied to dilate blood vessels and
reduce symptoms

Systemic Scleroderma
Nursing Interventions
Teach patient
Dress warmly, wear gloves and socks, and
avoid stress, any source of cold (including
AC and refrigeration)
Use moisturizers and oils to alleviate mild
skin irritation and dryness and to prevent
cracking
Systemic Scleroderma
Nursing Interventions
Teaching (cont.)
Avoid hot baths and showers
Use humidifiers to moisten air
Immunosuppressive drugs
NSAIDS, low-dose corticosteroids
Systemic Scleroderma
Nursing Interventions
Teaching (cont.)
Remain active
Use of articifial tears and good oral hygiene
Small, frequent meals, HOB elevated, tx acid
reflux
Quality of life issues
Appearance, mental health, sexuality, child-
bearing
Systemic Scleroderma
Sjgren's syndrome is a chronic
autoimmune disease in which a
persons white blood cells attack their
moisture-producing glands.
Today, as many as 4 million Americans
are living with this disease.
Nine out of 10 patients are women.
It affects about 1% of those with
scleroderma


Sjogrens syndrome
Manifestations
tear production causes gritty,
sandy, dry eyes with thick, ropy
secretions in inner canthus of the
eyes
Dry, erythematous sticky oral
mucosa

Sjogrens syndrome
Less frequent: lungs, throat, nose,
trachea, GI tract, vulva, and
vagina
S/S: easy fatigability, low-grade
fever, myalgias and arthralgias,
dyspnea
Pseudolymphoma from
generalized involvement of
lymph nodes, bone marrow, and
visceral organs


Sjogrens syndrome
Diagnostics
Salivary gland biopsy and flow
rate
Schirmers tear test
Sjogrens syndrome
Treatment is symptomatic
Artificial tears, saliva substitutes
Meticulous oral care
Corticosteroids & immunosuppressive
drugs for pseudolymphoma
Vaginal lubricants like KY jelly
Humidify the home
Sjogrens syndrome
Prevalence has grown by more than 30%
since the late 1970s
Changing sexual practices
Wider use of aggressive chemotherapies
for cancer
Immunosuppressive therapies for organ
transplantation

Herpes
Nearly 100 different herpes viruses
identified

Eight human types

Only influenza and cold viruses
cause more viral infections

Herpes
Herpes simplex
HSV-1: oral infections


HSV-2: genital infections


Herpes
Named from Greek word herpein to
creep Reflects the way the disease spreads
Not all cause lesions
Lifetime infection
Initial (primary) infection usually more severe
than recurrences

Herpes
Usually remains dormant or latent
Flares up and is reactivated due to
Stress, trauma, immunosuppression
Hormonal changes
Invasive procedures
Hospitalization
Weather changes

Herpes
Can be spread only when active viral
replication is occurring
During the primary infection
During a recurrent infection
Herpes
Fresh virus-containing body fluid from an infected
person must be directly inoculated onto tissues such
as oral, genital, or anal mucosa
Likelihood of transmission depends on the
quantity of virus shed
Quantity is higher during symptomatic infections
May have asymptomatic infections and can be
infective

Herpes
Cant survive for long periods of time outside
the host
Surfaces not actually contaminated with
body fluids pose minimal risk of infection
(i.e. toilet seats)
Herpes
HSV-1
HSV-1 (HHV-1)
Common cold sores and fever blisters
Encephalitis (less common)

Risk factors frequent intimate contact with an
infected person, including contact with mucosal
surfaces or abraded skin, and sharing eating
utensils, razors, and towels





Herpes Simplex
HSV-2 (HHV-2)
Genital herpes
Aseptic meningitis
?cervical cancers

Risk factors having multiple sex
partners and having other sexually
transmitted diseases

Herpes Simplex
Both HSV-1 and HSV-2
enter the body through breaks in
the skin and mucous membranes
can cause lesions in the facial
and/or genital areas

Herpes Simplex
dormant virus will periodically
reactivate at or near the original
site of infection
Fever, fatigue, menstruation,
stress, and illness can trigger the
reactivation. Exposure to sun
can also trigger recurrences

Herpes Simplex
can be transmitted even if lesions
are not visible, as long as
asymptomatic shedding of the
virus is occurring
the greatest risk of infection is from
the time a blister first appears until
its completely scabbed over.

Herpes Simplex
Treated with acyclovir (Zovirax, Avirax),
valacyclovir HCL (Valtrex), or famiciclovir
(Famvir)

Herpes Simplex
A painful infection of the hand by
HSV-1 or HSV-2
Can result from healthcare
workers contact with herpes
simplex on their unprotected
hands
Herpetic whitlow
Herpetic whitlow
HHV-3 or Varicella-zoster virus (VZV)

Varicella chicken-pox is the
primary infection
Herpes zoster Shingles is the
recurrent infection

Herpes Zoster/Varicella Zoster
Varicella Zoster
Chickenpox is preventable by use of the live-
attenuated varicella vaccine (Varivax),
recommended for children 12 months and older,
and susceptible adults
Varicella-zoster immune globulin (VZIG) effective
for short-term immunity if administered within 96
hours of exposure
Treated with Acyclovir (for CP or HZ)
Valacyclovir or famciclovir (for
Varicella Zoster
66% are older than 50yo; 5% less
than 15yo
Occurs in 10-20% of population

Herpes Zoster/ Shingles
Stress is a reactivation trigger

Immunocompromised patients
& the elderly
Painful, burning, localized rash in a
line pattern following the affected
nerve pathway

Herpes Zoster/ Shingles
HHV-4 Epstein-Barr virus (EBV)
Infectious mononucleosis
Associated with
Burkitts lymphoma
CNS lymphoma
Nasopharyngeal cancer
Hodgkins disease

Epstein-Barr virus (EBV)
Risk factors
frequent intimate contact, including saliva
on shared eating utensils
Symptoms
Fever, sore throat, swollen glands, and
fatigue that can be prolonged
Dormant EBV can reactivate after a
prolonged period and be shed, most
commonly from the throat in saliva


Epstein-Barr virus (EBV)
Supportive therapy
Steroids for patients with severe tonsillitis
that causes airway obstruction

Epstein-Barr virus (EBV)
HHV-5 Human cytomegalovirus (CMV)
Supportive therapy
For immunocompromised
Ganciclovir sodium (Cytovene)
Forcarnet socium (Foscavir)
Cidofovir (Vistide)

Human cytomegalovirus (CMV)
Symptoms fever, swollen glands, and
fatigue
Children in daycare centers
Transplant or blood recipients
Those with multiple sex partners
Can cause a mononucleosis-type illness in
some

Human cytomegalovirus (CMV)
Virus can occasionally be shed in urine or
saliva
Perinatal infection can occur from CMV
carried in the cervix during the late stages of
pregnancy and from CMV in breast milk
Direct contact with saliva, blood, urine,
breast milk, vaginal secretions, and semen

Human cytomegalovirus (CMV)
CMV - newborn
CMV - retinitis
HHV-6 and HHV-7
No common names
Closely related
Primary infection with either usually occurs
before age 3

HHV-6 & HHV-7
Usually asymptomatic
Mild, febrile illness called roseola
infantum, with high fever for 3 5 days
followed by a maculopapular red rash

HHV-6 & HHV-7
After primary infection, the virus replicates in
salivary glands and is shed in saliva
intermittently without symptoms
Reactivation may occur in
immunocompromised patients undergoing
organ transplant

HHV-6 & HHV-7
Herpes Virus
Herpes Virus
HHV-8 Kaposis sarcoma herpesvirus
(KSHV)
Human tumor virus
Closely linked to all forms of Kaposis
sarcoma, in addition to primary
effusion lymphoma and Castlemans
disease

Kaposis sarcoma
Kaposis sarcoma
Kaposis sarcoma
Establishes a latent infection, and it has the
potential to be reactivated under certain
conditions such as those caused by being
immunocompromised
Symptoms include skin lesions and tumors

Kaposis sarcoma
Sexual transmission, oral-anal contact,
organ transplant, possibly deep kissing
Treated with radiation, cytotoxic
chemotherapy, interferon-alpha

Kaposis sarcoma
Diagnosis
Made clinically for some (e.g. shingles)
Laboratory confirmation for genital herpes
(HSV-1 or -2) may exclude other illnesses,
assist with treatment options, and relieve
anxiety
Virus culture for HSV-1 and -2

Herpes Virus
Serology for antigen or antibody testing
Immunofluorescence assay
ELISA assay
Tzanck smear (examination of cells
scraped from the lower surface of a
vesicle) can be used as a quick test, but
cant differentiate between the eight virus
types

Herpes Virus
Infectious Diseases of
the Skin - Fungal

Tinea cruris
(Jock itch)

Tinea pedis
(Athletes foot)





Infectious Diseases of the Skin -
Bacterial
Folliculitis


Folliculitis
Furuncles (or boil)

Furuncles (or boil)

Cellulitis

Cellulitis

Cellulitis
Standard precautions
Used with all clients to protect against
transmission of organisms.


Airborne precautions (HEPA filter masks, etc.)
Used for clients with measles, chicken
pox/shingles, TB.


CDC Isolation Guidelines


Droplet precautions (Regular
masks)
Used for clients with influenza,
mumps, pertussis, meningitis.


Contact precautions
Used for clients with MRSA and
VRE, pediculosis, scabies, RSV, C-
difficile.
CDC Isolation Guidelines