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Case Report
Serum negative immunoglobulin G4-associated cholangiopathy mimicking hilar cholangiocarcinoma: A case report and review
1 Medical Student, College of Medicine, University College Hospital, Queen Elizabeth Road, P.O. Box 200212, Ibadan, Nigeria
2 Department of Medicine, University of Chicago Medicine, 5844 S. Maryland Avenue, Chicago, Illinois 60637, USA
Address correspondence to:
Chinedu Nwaduru
College of Medicine, University College Hospital, Queen Elizabeth Road, P.O. Box 200212, Ibadan,
Nigeria
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Article ID: 100087Z04CN2020
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Nwaduru C, Couri T, Pillai A. Serum negative immunoglobulin G4-associated cholangiopathy mimicking hilar cholangiocarcinoma: A case report and review. Int J Hepatobiliary Pancreat Dis 2020;10: 100087Z04CN2020.ABSTRACT
Introduction: Immunoglobulin G4-associated cholangiopathy (IAC) is a systemic manifestation of IgG4-related diseases manifesting with an increased serum level of IgG4. Immunoglobulin G4-associated cholangiopathy often has a robust clinical response to steroid therapy, however making a diagnosis can be difficult as the cholangiographic features may resemble that of cholangiocarcinoma with varying immunological profiles. In the absence of features suggestive of malignancy, a high index of suspicion for the disease should be maintained, even in the presence of normal serum levels of IgG4.
Case Report: In this case report, the diagnosis of IAC was made following cholangiographic imaging, along with the presence of retroperitoneal fibrosis and thickened urothelia, although serum IgG4 levels were within normal limits. The resolution of biliary strictures following a trial of steroid therapy further confirmed the diagnosis.
Conclusion: Immunoglobulin G4-associated cholangiopathy requires a combination of clinical, serological, histopathological, and radiological features in order to make a clear diagnosis. A trial of steroid therapy in the event of an unclear clinical presentation further helps in differentiating IAC from hilar cholangiocarcinoma.
Keywords: Autoimmunity, Cholangiocarcinoma, Immunoglobulin G4-associated cholangiopathy, Steroids
SUPPORTING INFORMATION
Acknowledgments
We thank John Fung, MD, PhD (Department of Surgery, University of Chicago) for his contributions and comments to the manuscript.
Author ContributionsChinedu Nwaduru - Substantial contributions to conception and design, Final approval of the version to be published
Thomas Couri - Revising it critically for important intellectual content, Final approval of the version to be published
Anjana Pillai - Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 Chinedu Nwaduru et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
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