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KMID : 0882420140870020182
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Korean Journal of Medicine
2014 Volume.87 No. 2 p.182 ~ p.186
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A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course
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Kim Tae-Oh
Kim Myung-Hwan
Choi Jun-Hyuk
Lim Doo-Ho
Park Sang-Woo
Choi Jun-Ho
Kim Jin-Hee
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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.
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KEYWORD
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Immunoglobulin G4, Sclerosing cholangitis, Cholangiocarcinoma
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