Internal Medicine

0521779407-C02 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:53

358 Churg Strauss Disease

➣Occult infection or malignancy

➣Myocardial ischemia

➣Wegener’s granulomatosis and polyarteritis nodosa

➣Central retinal artery occlusion, retinal detachment, glaucoma

➣Thyroid disease

➣Polymyositis, SLE, rheumatoid arthritis

➣Arterial fibrodysplasia

■WG, CS

➣Chronic sinusitis

➣Polyarteritis nodosa; microscopic polyangiitis

➣Fungal, bacterial diseases; syphilis

➣SLE

management

What to Do First

■Assess extent and severity of vascular disease by history and physical

exam; angiography especially in suspected TA

■Control blood pressure

■With impending organ damage, e.g. visual loss, evolving stroke, begin

immediate “pulse” (methylprednisolone, 1 gm/d qd×3).

■Ophthalmologic consultation in GCA, (eye disease often unilateral

initially, but may soon involve the other eye)

■ENT consultation in WG.

specific therapy

■GCA

➣prednisone is usually dramatically effective within 2–3 days.

Taper slowly

■TA, WG, CS

➣prednisone, tapering slowly over months.

➣cyclophosphamide orally

➣methotrexate may suffice in milder or controlled disease.

➣trimethoprim-sulfamethoxazole may suffice in patients with WS

confined to the upper airways; should be considered even in

systemic disease

➣other options include tumor necrosis factor-alpha antagonists,

mycophenolate mofetil, and azathioprine

Side Effects & Contraindications

■As in Nongranulomatous Vasculitis