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

p-ANCA가 동시에 검출된 항사구체 기저막 항체 질환 2예

  • 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)
  • 강건우 (계명대학교 의과대학 내과학교실) ;
  • 황은아 (계명대학교 의과대학 내과학교실) ;
  • 한승엽 (계명대학교 의과대학 내과학교실) ;
  • 박성배 (계명대학교 의과대학 내과학교실) ;
  • 김현철 (계명대학교 의과대학 내과학교실) ;
  • 최미선 (계명대학교 의과대학 병리학교실)
  • Published : 2012.10.01

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.

저자들은 폐출혈이 동반되지 않는 항사구체 기저막 항체 질환 환자에서 동시에 p-ANCA가 검출된 증례 2예를 경험하여 이를 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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