Anti-glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against the non collagenous (NC1) domain of the α3 chain of type 4 collagen (α3(IV)NC1). Clinical features are typically of a rapidly progressive glomerulonephritis (RPGN) with or without pulmonary haemorrhage. Treatment aims to rapidly remove circulating autoantibodies with plasma exchange and prevent further antibody production and suppress inflammation using immunosuppression and corticosteroids. Retrospective studies have shown that this combination of treatment results in good renal outcomes compared to historical controls. Disease relapse is uncommon and, unless patients have a co-existing antineutrophil cytoplasm antibody, maintenance treatment is not required.Le texte complet de cet article est disponible en PDF.