The Korean Journal of Medicine

  • 제83권6호
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  • Pages.807-812
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  • 2012
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  • 1738-9364(pISSN)
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  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
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P-ANCA 양성 항사구체기저막 항체 질환 1예

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease

  • 정현아 (건국대학교 의과대학 내과학교실) ;
  • 손인성 (건국대학교 의과대학 내과학교실) ;
  • 황용 (건국대학교 의과대학 내과학교실) ;
  • 최홍석 (건국대학교 의과대학 내과학교실) ;
  • 김도영 (건국대학교 의과대학 내과학교실) ;
  • 임소덕 (건국대학교 의과대학 병리과학교실) ;
  • 조영일 (건국대학교 의과대학 내과학교실)
  • Chung, Hyun Ah (Department of Internal Medicine, Konkuk University School of Medicine) ;
  • Son, In Sung (Department of Internal Medicine, Konkuk University School of Medicine) ;
  • Hwang, Yong (Department of Internal Medicine, Konkuk University School of Medicine) ;
  • Choi, Hong Seok (Department of Internal Medicine, Konkuk University School of Medicine) ;
  • Kim, Do Young (Department of Internal Medicine, Konkuk University School of Medicine) ;
  • Lim, So Duk (Department of Pathology, Konkuk University School of Medicine) ;
  • Jo, Young Il (Department of Internal Medicine, Konkuk University School of Medicine)
  • 발행 : 2012.12.01
⟨ 이전 논문 다음 논문 ⟩

초록

항사구체기저막 항체 질환은 드문 자가면역성 질환으로 급속 진행성 사구체신염으로 흔히 발현된다. 많게는 40%의 환자에서 ANCA가 양성인데, 이렇게 항사구체기저막 항체와 ANCA가 모두 양성인 경우에는 대체로 예후가 좋지 않다. 저자들은 p-ANCA가 양성인 항사구체기저막 항체 질환으로 투석이 필요했던 59세 여자 환자에서 혈장분리교환술과 적극적인 면역억제 치료로 신기능의 회복을 보여 투석치료를 중지한 급속 진행성 사구체신염 1예를 경험하였기에 문헌고찰과 함께 보고한다.

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.

키워드

참고문헌

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