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Case Report
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Korean J Med. 2014;87(2):182-186. Published online August 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.87.2.182
- 6년간의 자연 경과를 관찰할 수 있었던 IgG4 연관 경화성 담관염
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김태오1, 김명환1, 최준혁1, 임두호1, 박상우1, 최준호1, 김진희2
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1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 영상의학과
- 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
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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
- Corresponding author: Myung-Hwan Kim ,Tel: +82-2-3010-3183, Fax: +82-2-476-0824, Email: mhkim@amc.seoul.kr
- Received: December 26, 2013; Revised: January 10, 2014 Accepted: January 24, 2014.
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߽ɾ :Immunoglobulin G4; 경화담관염; 담관암종
- 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.
Keywords :Immunoglobulin G4, Sclerosing cholangitis, Cholangiocarcinoma
