A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic Antibody / 대한내과학회지

Anti-glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane. This disease accounts for 10-20% of crescentic glomerulonephritis. We report two patients with anti-GBM Ab disease who were positive for perinuclear-anti-neutrophil cytoplasmic antibody (p-ANCA). Percutaneous renal biopsies showed many crescent formations and linear deposits of IgG along the glomerular basement membrane. Serologic tests for p-ANCA were positive. They were treated with steroid pulse and cyclophosphamide and one patient also underwent plasma exchange therapy. Despite immunosuppressive therapy, their renal functions did not improve and both required regular hemodialysis.

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

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.