WEGENER'S GRANULOMATOSIS: CLINICAL FEATURES AND OUTCOME IN SEVENTEEN PATIENTS

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Abstract
Seventeen patients with Wegener''s granulomatosis are reviewed. Eleven males and 6 females, with a mean age of 46.9 .+-. 4.5 yr, were followed for 35.7 .+-. 9.0 mo. Mean duration from time of onset of symptoms to diangosis was 8.5 .+-. 3.1 mo. Constitutional symptoms (100%), lower respiratory tract involvement, (93%), renal involvement (87%) and upper respiratory tract involvement (93%) were the most frequent clinical manifestaitons. Arthiritis (60%), dermal vasculitis (60%) and inflammatory ocular disease (40%) were also common. Elevated ESR [erythrocyte sedimentation rate] (94%), anemia (70%) and lymphopenia (77%) were frequent laboratory findings prior to treatment. Five patients had renal failure at presentation and 2 patients progressed from no renal involvement of presentation to renal failure at diagnosis, while 5 patients progressed from renal involvement without impairment at diagnosis to end-stage renal failure. Seven patients died; 6 of these deaths were related to active Wegener''s granulomatosis. The patients with a severe systemic vasculitis and renal involvement had a poor outcome while predominant respiratory disease had a good prognosis.