Anti-glomerular basement membrane antibody disease: A rare autoimmune disorder affecting the kidney and the lung

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• 作者：Tobias Lahmer ; ^{Tobias.Lahmer@lrz.tu-muenchen.de} ; Uwe Heemann
• 关键词：Anti ; GBM disease ; RPGN ; Immunosuppression ; Goodpasture's disease ; Renal failure ; Pulmonary hemorrhage ; Plasmapherisis
• 刊名：Autoimmunity Reviews
• 出版年：2012
• 出版时间：December, 2012
• 年：2012
• 卷：12
• 期：2
• 页码：169-173
• 全文大小：802 K

文摘

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.