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KMID : 0882420120830040514
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Korean Journal of Medicine
2012 Volume.83 No. 4 p.514 ~ p.519
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Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic Antibody
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Kang Gun-Woo
Hwang Eun-Ah
Han Seung-Yeup
Park Sung-Bae
Kim Hyun-Chul
Choe Mi-Sun
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Abstract
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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.
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KEYWORD
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Anti-glomerular basement membrane antibody disease, Glomerulonephritis, Perinuclear-anti-neutrophil cytoplasmic
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