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Titlebook: IgG4-Related Sclerosing Cholangitis; Terumi Kamisawa,Myung-Hwan Kim Book 2019 Springer Science+Business Media Singapore 2019 IgG4.Primary

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Overview,t as a new clinical entity. IgG4-SC has a cholangiographic appearance similar to that of primary sclerosing cholangitis (PSC) and cholangiocarcinoma [1]. IgG4-SC respond well to steroid therapy (Fig. 1.1). In contrast, PSC is progressive and resistant to therapy, eventually involving both the intra-
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Pathology,ithelium left intact. Transmural inflammation with storiform fibrosis that gives rise to wall thickening is a highly typical histological finding, which may be solely sufficient for diagnosis. Involvement of the connective tissue surrounding the bile ducts is common, and if this involvement is emine
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Diagnostic Criteria,pancreatic cancer, and cholangiocarcinoma, the differential diagnosis of IgG4-SC from these three progressive or malignant diseases is very important. In the criteria of Japanese group, diagnosis of IgG4-SC is based on the following four criteria: characteristic biliary imaging findings, elevation o
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