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http://dx.doi.org/10.3904/kjm.2012.83.4.514

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

Kang, Gun-Woo (Department of Internal Medicine, Keimyung University School of Medicine)
Hwang, Eun-Ah (Department of Internal Medicine, Keimyung University School of Medicine)
Han, Seung-Yeup (Department of Internal Medicine, Keimyung University School of Medicine)
Park, Sung-Bae (Department of Internal Medicine, Keimyung University School of Medicine)
Kim, Hyun-Chul (Department of Internal Medicine, Keimyung University School of Medicine)
Choe, Mi-Sun (Department of Pathology, Keimyung University School of Medicine)
Publication Information
The Korean Journal of Medicine / v.83, no.4, 2012 , pp. 514-519 More about this Journal
Abstract
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.
Keywords
Anti-glomerular basement membrane antibody disease; Glomerulonephritis; Perinuclear-anti-neutrophil cytoplasmic antibody;
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