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KMID : 0882420120830060807
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Korean Journal of Medicine
2012 Volume.83 No. 6 p.807 ~ p.812
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A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease
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Chung Hyun-Ah
Son In-Sung
Hwang Yong
Choi Hong-Seok
Kim Do-Young
Lim So-Duk
Jo Young-Il
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Abstract
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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.
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KEYWORD
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Anti-glomerular basement membrane antibody disease, ANCA, Glomerulonephritis
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