A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma.
Publication/Presentation Date
12-17-2025
Abstract
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.
ISSN
2383-5001
Published In/Presented At
Hong, G., Hussain, A., Correia, E. T. O., Shalaby, A., Bittencourt, L. K., Mahipal, A., Ocuin, L. M., & Sclair, S. N. (2025). A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma. Journal of liver cancer, 10.17998/jlc.2025.12.09. Advance online publication. https://doi.org/10.17998/jlc.2025.12.09
Disciplines
Medicine and Health Sciences
PubMedID
41402032
Department(s)
Department of Surgery
Document Type
Article