Wegener's granulomatosis. Clinical features and outcome in 13 patients

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Abstract
Thirteen patients with Wegener''s granulomatosis were seen over 10.5 yr. Clinical features resembled those in previously reported series, except for the increased frequency of inflammatory arthritis, which was a prominent early feature in 10 patients (77%). Of the 13, 4 (31%) had fulminant vasculitis and died before receiving an adequate course of cytotoxic drug therapy. Of these 4, 2 had a pulmonary-renal syndrome that mimicked Goodpasture''s syndrome. All of the remaining 9 patients (69%) achieved an initial remission with cytotoxic agents (azathioprine or cyclophosphamide) but 4 died in less than 1 yr with no evidence of vasculitis at autopsy. The 56% survival rate to 1 yr in these 9 patients contrasts with an 86-100% survival in other series. Chronic renal failure was a prominent sequela in those who survived 1 yr.