IgG4-related sclerosing cholangitis is one of the major types of inflammation within the biliary tree and must be distinguished carefully from primary sclerosing cholangitis and other conditions. Typical cases of IgG4-related sclerosing cholangitis that accompany type 1 autoimmune pancreatitis or other classic organ system manifestations of IgG4-RD are well described. In addition, however, cases associated with atypical features, unusual concomitant diseases or syndromes, or malignancies have also been reported.
The finding of increased numbers of IgG4-positive plasma cells is not specific for IgG4-RD because such cellular infiltrates can be observed in multiple conditions, including cholangiocarcinoma. Important work in diagnostic criteria has been performed, but in the present state of knowledge, characterized by large gaps in understanding, diagnoses must often be made and treatment rendered on a case-by-case basis, with careful consideration of the clinical, serologic, radiologic, and pathological features; knowledge of disease in other organs; and close clinicopathological correlation.
Bile Duct Primary Sclerosing Cholangitis Autoimmune Hepatitis Sclerosing Cholangitis Bile Duct Injury
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