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| A case report of IgG4-related sclerosing cholangitis first presenting as jaundice without autoimmune pancreatitis |
| YANG Xianwen1 MA Zhiying2 |
1.Department of Gastroenterology, Liuzhou People’s Hospital, Guangxi Zhuang Autonomous Region, Liuzhou 545006, China;
2.Department of Hepatology, Liuzhou People’s Hospital, Guangxi Zhuang Autonomous Region, Liuzhou 545006, China |
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Abstract IgG4-related sclerosing cholangitis (IgG4-SC) is a unique type of sclerosing cholangitis characterized by significant increase in serum IgG4 level, infiltration of IgG4-positive plasma cells and lymphocytes, fibrosis of bile duct wall and obliterans phlebitis. IgG4-SC is often complicated with autoimmune pancreatitis (AIP), which is sensitive to steroid hormones Due to the low incidence of IgG4-SC and the lack of specific clinical manifestations, it is easy to miss diagnosis and misdiagnosis, and the recurrence rate is high after treatment. This paper reports a case of IgG4-SC with jaundice as the first manifestation and without AIP who received steroid therapy in Liuzhou People’s Hospital of Guangxi Zhuang Autonomous Region in December 2018. No recurrence was found during the follow-up of two years and nine months. To discuss the definition, clinical characteristics and significance of typical cholangiography in the diagnosis and treatment of IgG4-SC, and the application value of serum IgG4 level in the diagnosis and treatment of IgG4-SC, so as to improve the diagnosis and treatment of IgG4-SC with jaundice as the first manifestation without AIP.
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