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

Abstract

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.