A Case of Anti-Glomerular Basement Membrane Antibody Disease without Pulmonary Hemorrhage / 대한신장학회잡지

ABSTRACT

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis is a rare autoimmune disease. It is characterized by acuterenal failure and crescentic glomeruli with linear immune deposits along glomerular basement membrane mediated by anti-GBM antibodies. We report a case of a sixty-years-old man with generalized edema and hematuria. On admission, BUN/Creatinine was 118/19.6 mg/dL, Hb was 10.2 g/dL. On urinalysis, protein was 3+, and many RBCs were found. Renal biopsy specimen which contained 8 glomeruli showed active cellular crescent formation in all glomeruli. On immunofluorescent staining specimen, there were 4 glomeruli which showed strong IgG linear staining along the glomerular basement membrane and mild C3 & C1q deposit along the capillary walls. The titer of anti-GBM antibody was 123 EU by ELISA (normal <10 EU). We treated with high dose of corticosteroid and plasmapheresis, but renal function was not recovered even after 3 months of hemodialysis.