Atypical Noncrescentic Antiglomerular Basement Membrane Disease With?Concurrent Thin Basement Membrane?Nephropathy

摘要

Antiglomerular basement membrane (GBM) disease is caused by circulating autoantibodies against the noncollagenous domain of α3 chain of type IV collagen (α3NC1).1 Classic anti-GBM disease often presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage.2 Renal biopsy findings include crescentic GN on light microscopy and bright linear GBM IgG and C3 staining on immunofluorescence (IF).3 Atypical forms of anti-GBM disease can present as milder noncrescentic GN without pulmonary hemorrhage or in a pulmonary-predominant form without acute kidney injury, and diagnosis often relies on strong linear GBM staining for IgG and C3.3 It is a rare cause of acute GN with an estimated incidence of fewer than 1 case per million population.