Anti-Glomerular Basement Membrane Disease

Anti–glomerular basement membrane (GBM) disease often presents with rapidly progressive glomerulonephritis with or without lung hemorrhage. It is rare to present with lung hemorrhage alone. • Antibodies are generally directed at EA or EB epitopes found within the noncollagenous (NC1) domain of the α3 chain of type IV collagen. • Renal and patient survival in untreated anti-GBM is poor, although intensive therapy provides improved outcomes in patients with alveolar hemorrhage or rapidly progressive glomerulonephritis not requiring dialysis at outset. • Relapses are uncommon in patients with anti-GBM disease. • Patients who are double positive for antineutrophil cytoplasm antibody and anti-GBM have more relapsing disease and often need maintenance immunosuppressive therapy