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非典型Goodpasture病:一例临床病例报告及文献综述

Atypical Goodpasture's disease: a clinical case report and literature review.

作者信息

Bulanova M L, Potapov D V, Bulanov N M, Lysenko Kozlovskaya L V

机构信息

Vladimir Regional Clinical Hospital, Vladimir, Russia.

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

出版信息

Ter Arkh. 2018 Jun 20;90(6):130-136. doi: 10.26442/terarkh2018906130-136.

Abstract

Goodpasture's disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease.

摘要

肺出血肾炎综合征(抗肾小球基底膜病)是一种罕见的小血管血管炎,其特征是存在针对肾小球基底膜(GBM)和肺泡基底膜的自身抗体。抗GBM病的常见特征是快速进行性肾小球肾炎和肺泡出血(肺肾综合征)。我们报告一例年轻男性患者的非典型病程,该患者出现肺泡出血但无肾衰竭。肾脏受累的唯一症状是孤立性血尿。血浆置换联合免疫抑制(环磷酰胺和皮质类固醇)有效。我们对关于抗GBM病发病机制和病程的最新数据进行了综述。

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