The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course

6년간의 자연 경과를 관찰할 수 있었던 IgG4 연관 경화성 담관염

  • Kim, Tae-Oh (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Myung-Hwan (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Choi, Joon Hyuk (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lim, Doo-Ho (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Sangwoo (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Choi, Jun-Ho (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Jin Hee (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine)
  • 김태오 (울산대학교 의과대학 서울아산병원 내과) ;
  • 김명환 (울산대학교 의과대학 서울아산병원 내과) ;
  • 최준혁 (울산대학교 의과대학 서울아산병원 내과) ;
  • 임두호 (울산대학교 의과대학 서울아산병원 내과) ;
  • 박상우 (울산대학교 의과대학 서울아산병원 내과) ;
  • 최준호 (울산대학교 의과대학 서울아산병원 내과) ;
  • 김진희 (울산대학교 의과대학 서울아산병원 영상의학과)
  • Received : 2013.12.26
  • Accepted : 2014.01.24
  • Published : 2014.08.01
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Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare disease characterized by increased serum levels of IgG4 and dense infiltration of IgG4-positive plasma cells with fibrosis in the bile duct wall. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP) and typically shows an excellent response to steroid therapy. Despite recent progress in understanding the clinical presentation of IgG4-SC, its diagnosis still remains a challenge, particularly if it manifests as an isolated hilar stricture with normal serum IgG4 concentrations. In this article, we report the case of a 75-year-old man with IgG4-SC in whom it was difficult to distinguish hilar cholangiocarcinoma due to the normal serum IgG4 concentration and no accompanying AIP. He had an indolent clinical course and showed slow progression of a bile duct lesion over 6 years.

저자들은 간문부 담관암과 매우 유사하게 담도 협착을 보인 환자에서 지난 6년간의 임상 경과 및 영상 검사 소견을 기초로 담도 생검 및 스테로이드에 대한 치료 반응을 확인함으로써 IgG4-SC으로 확진할 수 있었던 증례를 문헌고찰과 함께 보고한다.

Keywords

References

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