Internal Medicine

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

Nongranulomatous Systemic Vasculitis 1057

➣Bilateral conjunctival injection, fever >5 days, rash, mucus mem-

brane changes (“strawberry tongue,” etc), cervical lymphade-

nopathy

tests

Lab Tests

■CBC (anemia, thrombocytosis)

■ESR and/or C-reactive protein elevated

■Urinalysis for glomerulonephritis, esp. in MPA, ECV

Other Tests

■Skin biopsy: necrotizing vasculitis w/ leukocytoclasis in most forms;

few or no immune deposits in MPA; IgA immune deposits in HSP;

cryoglobulin immune deposits in ECV

■Sural nerve biopsy (esp. in PAN, MPA): necrotizing inflammation of

small arteries

■ANCA: p-ANCA (anti-MPO) positive in MPA (40–80%)

■Cryoglobulins, rheumatoid factor in ECV

■Complement, ANA in some pts w/ LCV

■Hepatitis B, C, esp. in PAN, ECV

➣Blood cultures to exclude endocarditis

➣Antiphospholipid antibodies

■Other: In MPA, renal biopsy: necrotizing glomerulonephritis; pul-

monary capillaritis

Imaging

■Angiogram often shows aneurysms & vessel narrowing in PAN; coro-

nary aneurysms in 60% of pts who die of KD

■Echocardiography in CS: aortic root aneurysm

■Endoscopy in BD: mucosal ulcers, most often in right colon

■Chest X-ray: pulmonary infiltrates, hemorrhage; evaluate for emboli

in BD; infiltrates in MPA

■Audiogram (CS)

■Ophthalmologic exam (CS)

differential diagnosis

■Bacterial sepsis, especially endocarditis, also disseminated Neisse-

ria, rickettsiae

■Viral diseases, esp. hepatitis B, C

■Paraneoplastic syndromes assoc w/ cancers, esp. hematopoietic

malignancies & adenocarcinomas