Giant Cell Arteritis - Age >50 yrs

Characteristics Def: An acute and chronic granulomatous inflammation - nodular thickenings affected artery develop these nodular thickenings and may become thrombosed ischemia

Structures Involved Vasculitis produces this b/c thrombus Ischemia - Temporal artery (pain, head ache), - Ophthalmic arteryblindness, diplopia - Branches of external carotid arteries: TIA ( episodes last< 24 hours). Stroke (>24 hours)

Clinical Features - Polymyalgia rheumatica (joint & muscle pain), jaw pain (caludication), abrupt headaches, weight loss, anorexia, loss of vision and tenderness*. - Temporal headache, sudden diplopia.

Lab Diagnosis

Treatment

(palpably firm and painful)

Cannot see a thrombus heremaybe it is gone - ESR

Characteristics Takayasu Arteritis - Age <40 yrs - Female - pulseless disease - Granulomatous vasculitis

Structures Involved - branches of aortic arch, renal, pulmonary and Ophthalmic artery. - Renal artery Sec. hypertension ( Renin level)

Clinical Features 1. aneurysm 2. narrowing 3. Occlusion - BP and weaker pulses in the upper extremities ( carotid, radial, ulnar) than in the lower= pulseless disease*. - Ocular disturbances.

Lab Diagnosis

Treatment

Giant Cell

Characteristics Wegner Granulomastosis - aka Necrotizing (granulomatous) vasculitis Triangle of death 1. Hole in the hard palate (eroding the bone) systemic involvement 2. Giant cell granuloma 3. Renal and Respiratory system involved

Structures Involved - affects capillaries, venules, arterioles, and arteries.

Clinical Features - Giant cells is the blood vessels in lung, kidney - Death within 1 year if untreated (malignant course). - Nasopharynx: - Mucosal ulceration, sinusitis*, rupture of nasal septum/ bleeding. - Lung: Pneumonitis/condolidation= Hemoptysis*. 1. Mucosal ulcer in the nose 2. Sinusitis 3. Bleeding from nasal septum - Kidney: - Crescentic Glomerulonephritis acute renal failure (hematuria*+ elevated BUN, creatinine). - 20 Hypertension.

Lab Diagnosis - c-ANCA* is positive in the serum in 95% & is used to monitor disease activity.

dd TB bcz see caseous necrosis - giant cell seen in both but in this case it is found around the blood vessels NOT tissue Its a quick course.2-3 months

Characteristics Polyarteritis Nodosa (PAN) It is a Systemic vasculitis. Hepatitis B surface antigen present (HBsAg)*. Typically ANCA negative PAN produces aneurysm of the renal artery! Similar to that seen in the Wegenar.

Structures Involved - Involves Kidney, skin and other organs. Except the lungs***

Clinical Features Age: all age group Course: Episodic illness*. Others:1.Malaise, fever of unknown cause, and weight loss; 2.Hypertension, Abdominal pain and melena (bloody stool) 3.*Skin: Palpable skin Purpura ( but this is more common in leukocytoclastic vascuitis)

Lab Diagnosis Morphology: : Patchy fibrinoid necrosis (bright pink, fibrin-like, refractile intimal deposit)* of intima and adventitia with thrombosis.

Characteristics Features: Churg- Strauss Syndrome (variant of PAN)

Structures Involved

Clinical Features

Lab Diagnosis

PAN

Involve pulmonary vasculature Eosinophilia Asthma Morphologically is similar to PAN pANCA is positive MORPHOLOGY similar to PAN
Churg- Strauss Syndrome Eosinophilia No Kidney inv Involves LUNG pANCA pos.

No Eosinophilia Kidney inv. NO Lung pANCA neg.

Characteristics/ Etiology Immune Complex Etiology: reaction to an antigen Vasculitis: such as Drugs (e.g., penicillin), AKA (Microscopic Microorganisms (e.g., polyarteritis, streptococci) or Hypersensitivity, Malignancy OR Leukocytoclastic Vasculitis.) - pANCA is positive

Structures Involved Affects arterioles, capillaries, and venules-vessels. (venules like in Wegners) Deposition of antigen-antibody complex in vessels wall.

Clinical Features

Lab Diagnosis

Skin: Palpable skin Purpura ( this is more common than PAN), hemoptysis, arthralgia, muscle pain or weakness. Morphology: neutrophilic vasculitis*.

Characteristics/ Etiology HenochSchonlein Purpura - Type of Leukocytoclastic vasculitis -

Structures Involved IgA mediated Skin purpura (vasculitis). Dermal IgA deposit: Dermatitis herpetiformis. IgA deposition in kidney= recurrent hematuria. Abdominal manifestations: Pain, vomiting, and intestinal bleeding; nonmigratory arthralgia. Negative for panca.

Clinical Features

Lab Diagnosis

Characteristics/ Etiology Thromboangiitis Obliterance - AKA (Buerger Disease) Thrombus and abscess in the artery. Age & sex: middle aged( before 35 years) Men > women There is a very close association with smoking*. Tx Stop smoking!

Structures Involved Site: tibial and radial arteries.

Clinical Features

Lab Diagnosis

- Early: Fingers show Raynaud phenomenon and feet show claudication. - Late: Gangrene in some In upper limbs: Raynaud Phenomenon In lower limbs: Claudication Raynaud Phenomenon: Paroxysmal color change: whitebluered Two types: Primary = Vasoconstriction due to cold, stress etc= neurological. Secondary: due to pathological obstruction of artery as seen in Buerger disease, scleroderma, SLE etc.

Pathophysiology: Smoking Nicotine endothelial injury thrombus. --> ischemia gangreene (tibial/radial) Arrow is showing ABSCESS! (puss) - Lumen occluded by a thrombus with two microabscesses (arrow)

Characteristics/ Etiology Kawasaki Disease Age: young children

Structures Involved Location: coronary artery AneurysmMyocardial infarction

Clinical Features Clinical: conjunctivitis + maculopapular skin rash + lympadenopathy.

Lab Diagnosis

Looks like viral infection

Coronary artery aneurysm may occur.

Characteristics/ Etiology Systemic Lupus Erythematosus

Structures Involved

Clinical Features

Lab Diagnosis

Fibrinoid necrosis is present. Like PAN. Antibodies in SLE: Anticardiolipin Ab. deep VENOUS thrombosis (aka- Antiphospholipid syndrome) present. ds-DNA ANA, anti-smith ANA- not involved in DVT. Antiphospholipin syndrome of DVT 1. SLE, 2. DVT 3. Anticardiolipin Ab. - Fibrinoid necrosis seen here. IT is an exudate made out of fibrinogen.

Characteristics/ Etiology Varicose Veins Abnormally dilated, tortuous veins produced by 1.Prolonged, increased intraluminal pressure (valve incompetence). 2.Loss of vessel wall supportmuscle weakness- paralysis. 3.Weak valve or genetic

Structures Involved Esophageal varices: seen in patients who have cirrhosis of the liver and its attendant portal hypertension. Hemorrhoids: varicose dilation of the hemorrhoidal plexus of veins at the anorectal junction: produce fresh blood during defecation. Caused by repeated pregnancies, straining at stools (by cocaine).

Clinical Features Clinically silent (NO PAIN)

Lab Diagnosis

Varicos veine: without THROMBUS and is NOT painful/tender OF SUPERFICIAL VEINS (i.e great saphenus). Thrombophlebitis: vericose vein with THROMBUS and has Inflmmation and is TENDER and PAINFUL.

Characteristics/ Etiology Deep Vein Thrombosis Cause: these veins usually dont form thrombus b/c of the fast flow. Viscocity of the blood is elevated due to: hypercoagulable states: Adenocarcinoma , pregnancy, immobilization, bone fracture* and others. - Viscosity of the blood is elevated due to the

Structures Involved - Iliac, femoral, popliteal veins.

Clinical Features - All 3 veins: asymptomatic, leg swelling, erythema in some cases. Complications: Thrombus acute cor pulmonale (RHF). Also: Pulmonary infarction which is acute and quick death.

Lab Diagnosis

Note: thrombus. Infarcted area becomes RED.

Characteristics/ Etiology Angiosarcoma Tumor of the blood vessel Age: often in older adults Marker: CD31, CD34, or vWF, FVIII Carcinogen: Arsenic, polyvinyl chloride (plastic factory), Thorotrast.

Structures Involved - Most commonly in the SKIN and LIVER (others: soft tissue, breast).

Clinical Features -

Lab Diagnosis Morphology: proliferating vascular channels, hyperchromatic spindle cells (cancerous) and RBC.

Characteristics/ Etiology Kaposis Sarcoma Agent: HIV and KSHV-8 -If it occurs in the SKIN= Kaposis sarcoma. It is basically an angiosarcoma, of the skin. - HIV infectionImmune system is depressed herpes virus comes in to produce this sarcoma - Vimentin is a nonspecific marker here. Pathogenesis: KSHV proteins Incorporate in host genome and: 1.May disrupt the control of cellular proliferation (cell cycle S PHASE) by inactivating Rb gene , and 2.Prevent apoptosis of endothelial cells, through the production of p53 inhibitors.

Structures Clinical Features Involved - It is a dermal Clinical : Multiple growing red angiosarcoma. to purple skin plaques or nodules or on the hard palate (Kaposis does not look like a cancer clinically (despite it being cancer)it looks like a skin rash!!!. In perineal, lower limbs and oral cavity. NON-painful and does not look like cancer. Covers your whole skin very rapidly.

Lab Diagnosis Morphology: sheets of plump, proliferating hyper chromatic spindle cells & RBC (CD31, CD34 and vWF, Factor 4 and 8, Vimentin) and slit like spaces

Note: white slit like spaces. Lots of spaces & RBCs