Anti-Glomerular Basement Membrane Disease

Abstract

Anti–glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) with diffuse crescentic formation on renal biopsy, and it is a well-characterized cause of glomerulonephritis. In 1919, an autopsy of an 18-year-old male patient, who had developed hemoptysis and acute renal failure after experiencing flu-like symptoms, revealed massive alveolar hemorrhage, glomerulonephritis with fibrinous exudates in Bowman’s capsule and necrotizing vasculitis in the spleen and gut (Goodpasture, 1919). Stanton and Tange reported 9 cases with alveolar hemorrhage and RPGN as Goodpasture’s syndrome (Stanton & Tange, 1958). Anti-GBM disease was defined as the presence of serum autoantibodies to the noncollagenous domain of the alpha 3 chain of type IV collagen or a linear binding of IgG to glomerular capillary walls as detected by direct immunofluorescence in patients with RPGN. Anti-GBM disease was divided into two types: anti-GBM disease without alveolar hemorrhage was regarded as renal-limited anti-GBM disease, and that with alveolar hemorrhage was defined as Goodpasture’s syndrome. This review focuses on anti-GBM disease by comparing international differences in prevalence, clinical features, treatments and outcomes in order to improve the prognosis of anti-GBM disease.