IgG4-related sclerosing cholangitis: not always an obvious entity. 2021

Magdalini Velegraki, and Emmanouil Vardas, and Christos Dervenis, and Maria Fragaki, and Pinelopi Nikolaou, and Afroditi Mpitouli, and George Kazamias, and Athanasia Sepsa, and Elpida Giannikaki, and Gregorios A Paspatis
Department of Gastroenterology, Venizeleion General Hospital, Heraklion (Magdalini Velegraki, Emmanouil Vardas, Maria Fragaki, Pinelopi Nikolaou, Afroditi Mpitouli, Gregorios A. Paspatis).

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.

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