1.A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma
Gordon HONG ; Amber HUSSAIN ; Eduardo Thadeu de Oliveira CORREIA ; Akram SHALABY ; Leonardo K. BITTENCOURT ; Amit MAHIPAL ; Lee M. OCUIN ; Seth N. SCLAIR
Journal of Liver Cancer 2026;26(1):169-175
Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is a rare condition with symptoms often mimicking malignancy, infection, or other autoimmune diseases. This case report describes the unique case of a 62-year-old male initially diagnosed with IgG4-SC, followed by subsequent diagnosis of cholangiocarcinoma. Biliary tract cancer in the setting of IgG4 related disease has been previously described; however, this patient course is novel as it encompasses the spectrum of challenges in IgG4-SC management, including diagnostic uncertainty, risk of infection with immunosuppressive agents, and development of malignancy diagnosed shortly following IgG4-SC diagnosis. We review the literature of management, outcomes, and malignancy risk and furthermore, highlight a promising recent therapy in treatment of IgG4 related disease, inebilizumab.
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