Internal Medicine

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

1058 Nongranulomatous Systemic Vasculitis

➣Assoc w/ rheumatic disease (eg, SLE, rheumatoid arthritis, Sjo-

gren syndrome)

■Cholesterol, mycotic emboli

■Drug reactions

■Atrial myxoma

■Serum sickness

■Systemic amyloidosis

■Familial Mediterranean fever

management

What to Do First

■Assess extent & severity of vascular disease

■Control blood pressure; assessment of end-organ vascular integrity

■In cases of threatened organs (eg, abdominal angina w/ PAN, rapidly

progressive glomerulonephritis, CNS disease or rapidly progressive

mononeuritis multiplex, pulmonary disease; meningoencephalitis,

eye disease in BD; rapidly progressive hearing loss in CS), begin

“pulse” corticosteroids (eg, methylprednisolone, 1 g/d for 3 days)

■In MPA, ECV w/ severe progressive neuropathy, renal or severe pul-

monary disease, add cyclophosphamide, adjusting dose for WBC;

plasmapheresis sometimes also employed.

■In KD, aspirin or IV IgG, not corticosteroids

specific therapy

■Microscopic polyangiitis

➣Prednisone given initially & over the first 2–4 weeks then tapering;

cyclophosphamide may be necessary

■Polyarteritis nodosa, essential cryoglobulinemic vasculitis

➣As for MPA

➣Antiviral agents in hepatitis B, C-associated disease

■Cogan’s syndrome

➣Prednisone, w/ methotrexate added

■Limited cutaneous angiitis

➣Prednisone, w/ dapsone or cytotoxic agents

■Kawasaki’s disease

➣As outlined above under initial management

■Henoch-Schonlein purpura ̈

➣Most pts will do well w/ supportive care & NSAIDs

➣Corticosteroids may be required for nonsurgical GI problems &

cytotoxic agents for progressive renal disease

■Behc ̧et’s disease