| A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course |
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Tae-Oh Kim1, Myung-Hwan Kim1, Joon Hyuk Choi1, Doo-Ho Lim1, Sangwoo Park1, Jun-Ho Choi1, Jin Hee Kim2 |
1Departments of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Departments of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea |
| 6년간의 자연 경과를 관찰할 수 있었던 IgG4 연관 경화성 담관염 |
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김태오1, 김명환1, 최준혁1, 임두호1, 박상우1, 최준호1, 김진희2 |
1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 영상의학과 |
Correspondence:
Myung-Hwan Kim, Tel: +82-2-3010-3183, Fax: +82-2-476-0824, Email: mhkim@amc.seoul.kr
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Received: 26 December 2013 • Revised: 10 January 2014 • Accepted: 24 January 2014 |
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| This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
| Abstract |
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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. |
| Key Words:
Immunoglobulin G4; Sclerosing cholangitis; Cholangiocarcinoma |
| 주제어:
Immunoglobulin G4; 경화담관염; 담관암종 |
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