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Vasculitis Syndromes

Classification of Vasculitis Syndromes

The primary vasculitis syndromes are classified according to the size of the involved vessels:

Vasculitis of large arteries:

Giant cell arteritis of the aged (temporal arteritis)

Takayasu's arteritis

Vasculitis of medium and small vessels:

Classical polyarteritis nodosa

microscopic polyangiitis

infantile PAN (Kawasaki disease)

Wegener' s Granulomatosis

Vasculitis of small vessels:

drug-induced

Henoch-Schonlein purpura (HSP)

Essential mixed cryoglobulinemia

associated with vasculitis syndromes, vasculitis, giant cell arteritis, temporal arteritis, Takayasu's arteritis systemic rheumatic disease (rheumatoid arthritis, S.L.E., Sjogren's)

f. infection-related vasculitis syndromes, vasculitis, giant cell arteritis, temporal arteritis, Takayasu's arteritis

g. malignancy

h. urticarial vasculitis

B. Pseudo-vasculitic syndromes

1. Fever, skin rash, and a multitude of other symptoms and signs may be caused by infection.

2. Cholesterol atheroembolism

a. various cutaneous signs (a classic cause of "purple toes ^")

b. progressive renal failure

c. fever

d. elevated ESR

e. eosinophilia may occur

3. Atrial myxoma. Classic triad:

a. constitutional symptoms

b. embolic events

c. obstructive (hemodynamic vasculitis phenomena)

4. Antiphospholipid antibody syndrome

a. especially "catastrophic APA syndrome"

i. renal failure, HBP

ii. organ ischemia

iii. arterial & venous occlusions Vasculitis Syndromes, giant cell arteritis, temporal arteritis, Takayasu's arteritis

5. TTP. Classic pentad:

a. fever

b. thrombocytopenia

c. microangiopathic anemia

d. renal failure

e. fluctuating neurologic symptoms.