Epidemiology, Impact, and Management Strategies of Anti-Glomerular Basement Membrane Disease.

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Temporal associations with influenza, SARS-CoV-2 infection, and COVID-19 vaccination have been described although there is insufficient evidence to suggest causality. Anti-GBM disease accounts for approximately 20% of the cases of rapidly progressive GN cases secondary to crescentic GN, but is an uncommon cause of end-stage kidney disease. Early diagnosis by detection of circulating antibodies, increased awareness of atypical as well as complex clinical variants of the disease, and combined therapy with immunosuppression and plasma exchange has improved the prognosis of patients with this potentially fatal disease. Progress has been hampered by the rarity of anti-GBM disease, but new agents and therapeutic regimens are emerging.