As IgG4-related sclerosing cholangitis (IgG4-SC) exhibits various cholangiographic features similar to those of primary sclerosing cholangitis (PSC), 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 of serum IgG4 levels, coexistence of IgG4-related diseases, except those of the biliary tract, and characteristic histopathologic features. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP), and this association is useful for diagnosis of IgG4-SC. However, it is particularly difficult to accurately diagnose IgG4-SC in the absence of AIP. Evaluation of the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm a diagnosis of IgG4-SC.
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