Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2023 Nov 22;2023(11):rjad621.
doi: 10.1093/jscr/rjad621. eCollection 2023 Nov.

IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma

Wei R Ng  1 , Ngee-Soon Lau  2 , Mitali Fadia  3 , Sivakumar Gananadha  1
Affiliations
Case Reports

IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma

Wei R Ng et al. J Surg Case Rep. .

Abstract

A man in his 70s presented to the emergency department with painless obstructive jaundice. Initial blood test results show a predominantly cholestatic picture with elevated tumour markers, and imaging findings are concerning for a pancreatic head neoplasm or cholangiocarcinoma with involvement of the entire common bile duct. The patient underwent staging laparoscopy and biopsies including peritoneal washing, but did not identify any features of malignancy. Immunoglobulin G and immunoglobulin G4 testing were subsequently tested and shown to be elevated. The provisional diagnosis of immunoglobulin G4-related sclerosing cholangitis was made, and steroid treatment was empirically started. Treatment with steroids was successful, with complete resolution of symptoms and abnormal imaging findings and near complete resolution of liver function test results after 1 month.

Keywords: IgG4-related sclerosing cholangitis; autoimmune disease; cholangiocarcinoma.

PubMed Disclaimer

Conflict of interest statement

None declared.

Figures

Figure 1
Figure 1
CT scan showing thickening and enhancement of the entire extrahepatic common bile duct with intrahepatic duct dilatation.
Figure 2
Figure 2
MRCP showing involvement of entire common bile duct.
Figure 3
Figure 3
Abnormal FDG uptake on PET scan extending from intrahepatic duct to distal common bile duct with uptake in the tail of pancreas.
Figure 4
Figure 4
Follow-up MRCP with resolution of common bile duct pathology.
See this image and copyright information in PMC

References

    1. Li J, Zhao C, Shen Y. Autoimmune cholangitis and cholangiocarcinoma. J Gastroenterol Hepatol 2012;27:1783–9. - PubMed
    1. Kamisawa T, Okamoto A. IgG4-related sclerosing disease. World J Gastroenterol 2008;14:3948. - PMC - PubMed
    1. Culver EL, Webster G. IgG4-related sclerosing cholangitis. In: Hirschfield G, Adams D, Liaskou E (eds), Biliary Disease. Springer, Cham. 10.1007/978-3-319-50168-0_12. - DOI
    1. Kamisawa T, Okazaki, K. Diagnosis and treatment of IgG4-related disease. In: Okazaki K (eds), IgG4-Related Disease. Current Topics in Microbiology and Immunology, vol 401. Springer, Cham. 10.1007/978-3-319-50168-0_12. - DOI - PubMed
    1. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ. et al. . Immunoglobulin G4–associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008;134:706–15. - PubMed

Publication types