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김도영 ( Do Young Kim ),황용 ( Yong Hwang ),최홍석 ( Hong Seok Choi ),조영일 ( Young Il Jo ),임소덕 ( So Duk Lim ),손인성 ( In Sung Son ),정현아 ( Hyun Ah Chung ) 대한내과학회 2012 대한내과학회지 Vol.83 No.6
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. (Korean J Med 2012;83:807-812)