[Review on anti-glomerular basement membrane disease or Goodpasture's syndrome]

Abstract

Anti-glomerular basement membrane (anti-GBM) disease or Goodpasture's syndrome is a small vessel vasculitis affecting the capillary beds of kidneys and lungs. It is an autoimmune disease mediated by autoantibodies targeting the glomerular and alveolar basement membranes, leading to pneumorenal syndrome. It is a rare, monophasic and severe disease, associating rapidly progressive glomerulonephritis and alveolar hemorrhage. The presence of antineutrophil cytoplasmic antibodies (ANCA) is reported in 20 to 60% of cases. Management should be prompt and combine plasma exchange with systemic corticosteroids and immunosuppressive therapy by cyclophosphamide. The objective of this review is: 1) to describe the pathogenesis, clinical and histological features of the disease; 2) to characterize double-positive anti-GBM/ANCA patients; 3) to highlight the prognostic factors of renal and global survival, and 4) to focus on the treatment of anti-GBM disease.

Keywords: Anti-GBM; Anti-GBM disease; Anti-MBG; Anti-glomerular basement membrane antibodies; Anticorps anti-membrane basale glomérulaire; Goodpasture's syndrome; Maladie des anticorps anti-membrane basale glomérulaire; Syndrome de Goodpasture; Vascularite; Vasculitis.