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表现为肺出血肾炎综合征临床特征的韦格纳肉芽肿。

Wegener's granulomatosis exhibiting the clinical features of Goodpasture's syndrome.

作者信息

Boros G, Orbán I, Nagy J

出版信息

Int Urol Nephrol. 1981;13(4):375-85. doi: 10.1007/BF02081939.

Abstract

A case is reported here, the clinical features of which raised the suspicion of Goodpasture's syndrome which was, however, at variance with the absence of antiglomerular basement membrane antibodies. Renal failure improved on immunosuppressive treatment and peritoneal dialysis, but the patient died of gastric haemorrhage from a peptic ulcer related to steroid treatment. Necropsy and microscopic study revealed abnormalities of liver and spleen, consistent with Wegener's granulomatosis. Immunohistologic studies of the kidney confirmed the presence of granular-type IgG deposits in the glomeruli. There were extensive fibrin deposits in the kidney. The ways and means for the differentiation of Wegener's granulomatosis from Goodpasture's syndrome, the potential pathogenetic role of immunocomplexes and fibrin deposits, and the therapeutic possibilities are discussed.

摘要

本文报告了一例病例,其临床特征引发了对古德帕斯彻综合征的怀疑,然而,这与缺乏抗肾小球基底膜抗体的情况不符。免疫抑制治疗和腹膜透析使肾衰竭有所改善,但患者死于与类固醇治疗相关的消化性溃疡引起的胃出血。尸检和显微镜检查显示肝脏和脾脏存在异常,符合韦格纳肉芽肿病。肾脏的免疫组织学研究证实肾小球中存在颗粒型IgG沉积物。肾脏中有广泛的纤维蛋白沉积物。讨论了韦格纳肉芽肿病与古德帕斯彻综合征的鉴别方法、免疫复合物和纤维蛋白沉积物的潜在致病作用以及治疗可能性。

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