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Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the multisystem IgG4-related disease. IgG4-SC presents with biliary strictures and/or masses that can bear a striking similarity to other malignant and inflammatory diseases. Diagnosis is based on a combination of clinical, biochemical, radiological, and histologic findings with careful exclusion of malignant disease. Corticosteroids are the mainstay of treatment with good clinical, biochemical, and radiological responses. This review provides a comprehensive overview of the current knowledge of the prevalence, clinical features, radiology and histology findings, diagnosis, treatment, natural history, and pathophysiology of IgG4-SC.

Original publication




Journal article


Clin Liver Dis

Publication Date





47 - 65


Autoimmune pancreatitis, IgG4-associated cholangitis, IgG4-related disease, IgG4-related sclerosing cholangitis, Autoimmune Diseases, Chemokines, Cholangitis, Sclerosing, Humans, Immunoglobulin G, Prognosis, T-Lymphocytes, Helper-Inducer, T-Lymphocytes, Regulatory