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ARTICLE |

Wegener's Granulomatosis:  Clinical Features and Outcome in 13 Patients

Sydney Brandwein, MD; John Esdaile, MD; Deborah Danoff, MD; Hyman Tannenbaum, MD
Arch Intern Med. 1983;143(3):476-479. doi:10.1001/archinte.1983.00350030090016.
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• Thirteen patients with Wegener's granulomatosis were seen over 10.5 years. The clinical features resembled those in previously reported series, except for the increased frequency of inflammatory arthritis, which was a prominent early feature in ten patients (77%). Four (31%) of the 13 had fulminant vasculitis and died before receiving an adequate course of cytotoxic drug therapy. Two of these four had a pulmonary-renal syndrome that mimicked Goodpasture's syndrome. All of the remaining nine patients (69%) achieved an initial remission with cytotoxic agents (azathioprine or cyclophosphamide), but four died in less than one year with no evidence of vasculitis at autopsy. The 56% survival rate to one year in these nine patients contrasts with an 86% to 100% survival in other series. Chronic renal failure was a prominent sequela in those who survived one year.

(Arch Intern Med 1983;143:476-479)

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