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small vessel vasculitis
Etiology:
1) primary small vessel vasculitis (15-20%)
a) Wegener's granulomatosis (granulomatosis with polyangiitis)
b) Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis)
c) microscopic polyangiitis
d) Henoch-Schonlein purpura (IgA vasculitis)
e) leukocytoclastic vasculitis
f) cryoglobulinemia
g) Behcet's syndrome
h) Cogan's syndrome
2) connective tissue disease
a) systemic lupus erythematosis
b) rheumatoid arthritis
c) Sjogren's syndrome
3) inflammatory bowel disease
4) infections (15-20%)
a) group A beta-hemolytic streptococci
b) hepatitis B virus
c) hepatitis C virus (cryoglobulinemia)
d) septic vasculitis
5) pharmaceutical agents (10-15%)
a) sulfonamides
b) penicillins
c) allopurinol
d) phenytoin
e) thiazides
f) fluoroquinolones
6) malignancy (< 5%)
a) paraproteinemia
b) lymphoproliferative disease
7) idiopathic (50%)
Clinical manifestations:
- palpable purpura
Laboratory:
- full thickness skin biopsy
Differential diagnosis:
- primary small vessel vasculitis
- IgA vasculitis
- serum complement (C3,C4) normal, ANCA negative, hematuria, no proteinuria, leukocytosis with normal differential, abdominal pain, GI bleed, arthritis
- cryglobulinemia
- serum complement low, ANCA negative, cutaneous symptoms 100%, palpable purpura, Raunaud's phenomenon 50%
- ANCA-associated vasculitis
- granulomatosis with polyangiitis (Wegener's granulomatosis)
- eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- asthma, sinusitis, eosinophilia, ANCA positive in 50%
- microscopic polyangiitis
- pANCA, arthralia, myalgia, neuropathy, glomerulonephritis, hemoptysis from pulmonary vasculitis
- polyarteris nodosa
- skin nodules, skin ulcers (deeper than palpable purpura), myalgia, arthralgia, fever, weight loss, intestinal ischemia, testicular pain
Management:
1) identify underlying etiology
2) remove offending agents
3) sparing use of glucocorticoids
4) cutaneous small vessel vasculitis (palpable purpura) can be managed with
- rest & elevation of affected region
- topical glucocorticoids
- NSAIDs
- antihistamines
4) see specific etiology
Related
large vessel vasculitis (large vessel arteritis)
medium vessel vasculitis
Specific
acute leukocytoclastic vasculitis (Henoch-Schonlein purpura; IgA vasculitis)
allergic angiitis & granulomatosis of Churg-Strauss; eosinophilic granulomatosis with polyangiitis (EGPA)
Behcet syndrome
Cogan's syndrome; diffuse interstitial keratitis
cryoglobulinemia (cryoglobulinemic vasculitis)
cutaneous lupus erythematosus
granulomatosis with polyangiitis; Wegener's granulomatosis
leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome
microscopic polyangiitis
General
vasculitis
References
- Weiner S. In: Intensive Course in Geriatric Medicine &
Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- Medical Knowledge Self Assessment Program (MKSAP) 14, 15
American College of Physicians, Philadelphia 2006, 2009