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A case of IgG4-associated cholangitis and autoimmune pancreatitis responsive to corticosteroids

Nature Clinical Practice Gastroenterology & Hepatology volume 5, pages 707–712 (2008)Cite this article

Abstract

Background A 72-year-old male presented to the emergency department with epigastric pain, anorexia and progressive jaundice of 1 week's duration. He had no prior history of gastrointestinal illness, diabetes or cancer. He did not smoke or consume alcohol. He did have a family history of colon and bone cancer.

Investigations Biochemical and serologic studies, CT scan, abdominal ultrasound, endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, biliary cytology, pancreas needle biopsies and immunohistochemical stainings.

Diagnosis Autoimmune pancreatitis with IgG4-associated sclerosing cholangitis affecting the extrahepatic biliary ducts and mimicking primary sclerosing cholangitis and cholangiocarcinoma.

Management Corticosteroids and immunomodulatory therapy.

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Figure 1: Abdominal CT images of the patient are suggestive of autoimmune pancreatitis.
Figure 2: Needle biopsy sample from the case patient's pancreas.
Figure 3: IgG4 immunohistochemical staining of pancreatic needle biopsy tissue.
Figure 4: Cholangiogram obtained by endoscopic retrograde cholangiopancreatography shows a 4 cm stricture in the patient's common hepatic duct (arrow).
Figure 5: Follow-up cholangiogram after 8 weeks of corticosteroid therapy demonstrates complete resolution of strictures.

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Acknowledgements

Charles P Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. AJ Small is a Resident in Internal Medicine at New York-Presbyterian Hospital/Weill Cornell Medical College, NY.,

    Aaron J Small

  2. CG Loftus is a Consultant in the Division of Gastroenterology and Hepatology, TC Smyrk is an Associate Professor of Pathology in the Department of Anatomic Pathology and Laboratory Medicine, Division of Gastroenterology and Hepatology and Director of Pancreaticobiliary Endoscopy, and TH Baron is Professor of Medicine, at Mayo Clinic, Rochester, MN, USA.,

    Conor G Loftus, Thomas C Smyrk & Todd H Baron

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Correspondence to Todd H Baron.

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Small, A., Loftus, C., Smyrk, T. et al. A case of IgG4-associated cholangitis and autoimmune pancreatitis responsive to corticosteroids. Nat Rev Gastroenterol Hepatol 5, 707–712 (2008). https://doi.org/10.1038/ncpgasthep1296

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