Abstract
BackgroundIgG4-mediated sclerosing cholangitis (IgG4-SC) is an autoimmune disease that generally occurs in association with type 1 autoimmune pancreatitis (AIP). However, an isolated variant of IgG4-SC is a rare disease that can occur without concurrent AIP and closely mimics extrahepatic cholangiocarcinoma both clinically and radiologically.Case presentationHere, we report a case of an isolated variant of IgG4-SC, which was pre-operatively diagnosed as hilar cholangiocarcinoma. Pathological and immunohistochemical examination revealed features suggestive of IgG4-SC, which was confirmed by elevated serum IgG4 levels measured postoperatively.ConclusionTherefore, IgG4-SC should be carefully ruled out in patients presenting with isolated bile duct obstruction, before performing morbid surgical procedures.
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