Abstract
Background: IgG4-related sclerosing cholangitis (IgG4-SC) is a rare immune-mediated disorder affecting the biliary tree and leading to biliary strictures that may resemble cholangiocarcinoma (CCA).
Case Presentation: An elderly man presented with a one-week history of jaundice, pale stool, and dark urine. This was associated with unintentional weight loss over the past year. Laboratory findings were notable for cholestasis and significantly elevated CA19-9. Imaging demonstrated multiple diffuse biliary strictures of malignant appearance. Cytology showed atypical glandular cells. The patient was misdiagnosed with CCA and was assessed for a possible surgical intervention or chemoradiotherapy. However, the patient was eventually diagnosed with IgG4-SC after a high serum IgG4 level and liver biopsy. The patient responded well to steroids.
Conclusion: This case underscores the diagnostic dilemma posed by IgG4-SC, particularly in elderly patients with cholestatic jaundice and elevated CA 19-9, which can be misleading for malignancy.
Recommended Citation
Abbarh, Shahem; Sawaf, Bisher; Vij, Radhika; Oshima, Kiyoko; Kinowaki, Keiichi; and Sankineni, Abhinav
(2026)
"IgG4-Related Sclerosing Cholangitis Masquerading as Cholangiocarcinoma: A Diagnostic Challenge,"
Journal of Community Hospital Internal Medicine Perspectives: Vol. 16:
Iss.
2, Article 8.
DOI: 10.55729/2000-9666.1590
Available at:
https://scholarlycommons.gbmc.org/jchimp/vol16/iss2/8
DOI
10.55729/2000-9666.1590
