Department of Nephrology, Klinikum rechts der Isar, Technische Universitaet Muenchen, Germany.
Autoimmun Rev. 2012 Dec;12(2):169-73. doi: 10.1016/j.autrev.2012.04.002. Epub 2012 Apr 23.
Anti-glomerular basement membrane antibody disease is a rare, but well characterized cause of glomerulonephritis. By definition serum anti-GBM antibody and/or a linear binding of IgG detected by direct immunofluorescence (IF) in a histological specimen of the kidney or the lung have to be detected. These antibodies can lead to acute rapid progressive glomerulonephritis(RPGN) and/or pulmonary hemorrhage (PH) because of collagen similarities in the basement membrane. Principally anti-GBM antibody disease can be divided into two groups: anti-GBM antibody disease without PH was regarded as renal-limited anti-GBM antibody disease and that with PH was defined as Goodpasture's syndrome (GPS). The important determinant for the response of therapy and long term diagnosis on anti-GBM disease is early diagnosis to prevent endstage renal disease. Therefore, standard treatment is a combined therapy of plasmapherisis, prednisolone and cyclophosphamide. The aim of this review is an overview of the pathogenesis, clinical presentation, diagnosis and treatment of anti-GBM disease.
抗肾小球基底膜抗体病是一种罕见但特征明确的肾小球肾炎病因。根据定义，血清抗肾小球基底膜抗体和/或直接免疫荧光（IF）在肾脏或肺部组织学标本中检测到的线性 IgG 结合必须被检测到。这些抗体可导致急性快速进行性肾小球肾炎（RPGN）和/或肺出血（PH），因为基底膜中存在胶原相似性。原则上，抗肾小球基底膜抗体病可分为两组：无 PH 的抗肾小球基底膜抗体病被视为肾局限性抗肾小球基底膜抗体病，有 PH 的则定义为 Goodpasture 综合征（GPS）。治疗反应和长期诊断抗肾小球基底膜病的重要决定因素是早期诊断以预防终末期肾病。因此，标准治疗是血浆置换、泼尼松龙和环磷酰胺的联合治疗。本综述的目的是概述抗肾小球基底膜病的发病机制、临床表现、诊断和治疗。
