title: Immunoglobulin G4-mediated sclerosing cholangitis as a risk factor for cholangiocarcinoma: A case report.
abstract: Immunoglobulin (Ig)G4-mediated disease is a systemic autoimmune disease, which occasionally presents solely as sclerosing cholangitis (SC). IgG4-mediated SC is challenging to diagnose, as it may mimic cholangiocarcinoma radiologically, and carcinoma cells may produce IgG4. The diagnosis of IgG4-mediated disease is based on histological consensus criteria and response to corticosteroids. In addition to the radiological and histological overlap between IgG4-mediated SC and cholangiocarcinoma, IgG4-mediated SC may be considered as a risk factor for the development of cholangiocarcinoma. We herein present the case of a patient in whom cholangiocarcinoma developed in two lesions previously characterized as IgG4-mediated SC, including a suggested mechanism underlying the contribution of IgG4-mediated SC to the development of cholangiocarcinoma.
authors.affiliation: Department of Gastroenterology, Northwest Clinics, 1815 JD Alkmaar, The Netherlands.
authors.forename: Karin E
authors.initials: KE
authors.lastname: Koopman
authors.affiliation: Department of Pathology, VU University Medical Center (VUMC), 1081 HV Amsterdam, The Netherlands.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bloemena
authors.affiliation: Department of Surgery, VU University Medical Center (VUMC), 1081 HV Amsterdam, The Netherlands.
authors.forename: Geert
authors.initials: G
authors.lastname: Kazemier
authors.affiliation: Department of Gastroenterology, Northwest Clinics, 1815 JD Alkmaar, The Netherlands.
authors.forename: Michael
authors.initials: M
authors.lastname: Klemt-Kropp
keywords: cholangiocarcinoma; immunoglobulin G4-mediated disease; immunoglobulin G4-mediated sclerosing cholangitis