Internal Medicine

0521779407-15 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:43

1056 Nongranulomatous Systemic Vasculitis

NONGRANULOMATOUS SYSTEMIC VASCULITIS

ERIC L. MATTESON, MD

history & physical

■Polyarteritis nodosa (PAN)

➣Pts of any age; no gender difference

➣Fever, weight loss, hypertension, abdominal pain, peripheral

neuropathy, vasculitic skin rash, abnormal kidney function or

urine sediment due to renal ischemia or infarction, microa-

neurysms in visceral arteries

■Microscopic polyangiitis (MPA)

➣Like classic PAN but w/ renal disease w/ active sediment due to

necrotizing glomerulonephritis

➣Cough (w/ hemoptysis)

■Essential cryoglobulinemic vasculitis (ECV)

➣Similar to MPA; often w/ Raynaud’s phenomenon, livido reticu-

laris

■Cutaneous leukocytoclastic angiitis (LCA)

➣New-onset purpuric rash confined to skin, often w/ other rheu-

matic disease or drug exposure

➣No systemic involvement

■Behc ̧et’s disease (BD)

➣Male:female 2:1; mean age about 30 yrs

➣More common in pts of Asian or Mediterranean descent

➣Aphthous oral (100%) and/or genital (70%) ulcers, uveitis (75%),

meningoencephalitis 20%, arthritis (usually oligoarticular occa-

sionally sacroiliitis) (40%), cutaneous vasculitis (50%)

■Cogan’s syndrome (CS)

➣Interstitial keratitis (scleritis, uveitis, other forms of ocular

inflammation); visual disturbance, hearing loss arteritis, aorti-

tis, renal disease, abdominal pain

■Henoch-Schonlein purpura (HSP) ̈

➣Children of any age; adults less often

➣Peak incidence in spring & fall

➣Upper respiratory infection in preceding 1–3 weeks

➣Palpable purpura, especially lower extremities (100%)

➣Abdominal pain (70%), mild renal abnormalities (40%)

■Kawasaki’s disease (KD)

➣Children generally <8 yrs (usually <4 yrs) of age