Wegener's granulomatosis. Clinical features and outcome in 13 patients.
Wegener's granulomatosis. Clinical features and outcome in 13 patients.
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韦格纳肉芽肿病。
DOI:
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发表时间:
1983
影响因子:
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通讯作者:
H. Tannenbaum
中科院分区:
文献类型:
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作者:
S. Brandwein;J. Esdaile;D. Danoff;H. Tannenbaum
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.