IgG4-related sclerosing cholangitis: not always an obvious entity

Magdalini Velegraki; Emmanouil Vardas; Christos Dervenis; Maria Fragaki; Pinelopi Nikolaou; Afroditi Mpitouli; George Kazamias; Athanasia Sepsa; Elpida Giannikaki; Gregorios A Paspatis

doi:10.20524/aog.2021.0610

IgG4-related sclerosing cholangitis: not always an obvious entity

Ann Gastroenterol. 2021 Jul-Aug;34(4):594-596. doi: 10.20524/aog.2021.0610. Epub 2021 Feb 26.

Affiliations

¹ Department of Gastroenterology, Venizeleion General Hospital, Heraklion (Magdalini Velegraki, Emmanouil Vardas, Maria Fragaki, Pinelopi Nikolaou, Afroditi Mpitouli, Gregorios A. Paspatis).
² Hepatobiliary and Pancreatic Surgery Clinic, Metropolitan Hospital, Athens (Christos Dervenis).
³ Department of Histopathology, Venizeleion General Hospital, Heraklion (George Kazamias, Elpida Giannikaki).
⁴ Department of Histopathology, Metropolitan Hospital, Athens (Athanasia Sepsa), Greece.

Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a distinct type of cholangitis, currently recognized as a biliary manifestation of IgG4-related disease. We present a case of type 3 IgG4-SC in a patient with normal IgG4 serum levels, surgically treated for suspicion of cholangiocarcinoma. This case highlights that differentiating between isolated IgG4-SC and cholangiocarcinoma can present a challenging diagnostic dilemma.

Keywords: IgG4-related sclerosing cholangitis; cholangiocarcinoma.

Publication types

Case Reports