6³â°£ÀÇ ÀÚ¿¬ °æ°ú¸¦ °üÂûÇÒ ¼ö ÀÖ¾ú´ø IgG4 ¿¬°ü °æȼº ´ã°ü¿°
A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course
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±èÅ¿À(Kim Tae-Oh) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°úÇб³½Ç
±è¸íȯ(Kim Myung-Hwan) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°ú
ÃÖÁØÇõ(Choi Jun-Hyuk) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°ú
ÀÓµÎÈ£(Lim Doo-Ho) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°ú
¹Ú»ó¿ì(Park Sang-Woo) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°ú
ÃÖÁØÈ£(Choi Jun-Ho) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ³»°ú
±èÁøÈñ(Kim Jin-Hee) - ¿ï»ê´ëÇб³ ÀÇ°ú´ëÇÐ ¼¿ï¾Æ»êº´¿ø ¿µ»óÀÇÇаú
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.
Å°¿öµå
Immunoglobulin G4, Sclerosing cholangitis, Cholangiocarcinoma
KMID :
0882420140870020182
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