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KMID : 0985520170220040179
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Korean Journal of Pancreas and Biliary Tract
2017 Volume.22 No. 4 p.179 ~ p.183
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IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis
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Han Ji-Soo
Jun Jae-Hyuck
Jung Kyung-Hwa
Jeong Dae-Hyun
Park Jae-Cheol
Noh Soo-Min
Hsing Li-Chang
Kim Myung-Hwan
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Abstract
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Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive IgG4-positive plasma cells infiltration resulting in inflammation and fibrosis of the affected organs. Patient with IgG4-RD shows an excellent response to glucocorticoid therapy especially when given at early onset stages. IgG4-related sclerosing cholangitis (IgG4-SC) is considered biliary manifestation of IgG4-RD. The natural history and long-term prognosis of IgG4-SC is not well defined and most of the previous case series have reported short duration of follow-up. We present the case of development of decompensated liver cirrhosis from IgG4-related sclerosing cholangitis with a 6-year natural clinical course.
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KEYWORD
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Immunoglobulin G4, Hepatic cirrhosis, Cholangitis
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