Immunoglobulin G4-mediated sclerosing cholangitis as a risk factor for cholangiocarcinoma: A case report

Koopman,Karin   E.; Bloemena,Elisabeth; Kazemier,Geert; Klemt‑Kropp,Michael

doi:10.3892/mco.2016.1040

Immunoglobulin G4-mediated sclerosing cholangitis as a risk factor for cholangiocarcinoma: A case report

Authors:
- Karin E. Koopman
- Elisabeth Bloemena
- Geert Kazemier
- Michael Klemt‑Kropp
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Affiliations: Department of Gastroenterology, Northwest Clinics, 1815 JD Alkmaar, The Netherlands, Department of Pathology, VU University Medical Center (VUMC), 1081 HV Amsterdam, The Netherlands, Department of Surgery, VU University Medical Center (VUMC), 1081 HV Amsterdam, The Netherlands
Published online on: September 30, 2016 https://doi.org/10.3892/mco.2016.1040
Pages: 786-788

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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.

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