International Journal of Case Reports and Images - IJCRI - Case Reports, Case Series, Case in Images, Clinical Images

   
Case Report
 
Unmasking IgG4-related autoimmune pancreatitis from pancreatic cancer: A lesson learned
Ashwad Afzal1, Seema Chittalae1, Ivan Wong1, Petros Efthimiou1
1MD, Department of Internal Medicine, New York Methodist Hospital affiliate of Weill Medical College of Cornell University, Brooklyn, New York, USA.

doi:10.5348/ijcri-201620-CR-10607

Address correspondence to:
Ashwad Afzal
New York Methodist Hospital
Affiliate of Weill Medical College of Cornell University 506 6th street
Brooklyn, NY 11215
USA

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How to cite this article
Afzal A, Chittalae S, Wong I, Efthimiou P. Unmasking IgG4-related autoimmune pancreatitis from pancreatic cancer: A lesson learned. Int J Case Rep Images 2016;7(2):123–126.


Abstract
Introduction: Autoimmune pancreatitis (AIP) is difficult to distinguish from pancreatic cancer. An AIP has a similar clinical presentation and is one component of a systemic disease, Immunoglobulin G4 (IgG4) related sclerosing disease. IgG4 related sclerosing disease is characterized by extensive IgG4 positive plasma cells and T-lymphocytes that may involve the pancreas and other organs.
Case Report: A 77-year-old male presented with nausea, vomiting, decreased appetite, and 39 lbs weight loss over three months. An endoscopic ultrasound showed one 3.3x2.7 cm mass on the tail of the pancreas extending towards the splenic hilum with no lymph node involvement. A fine needle aspiration was indeterminate. The patient underwent distal pancreatectomy and splenectomy for presumed pancreatic cancer, however, the pathology report was negative for malignancy. Instead, the microscopic examination revealed dense lymphoplasmacytic infiltrate staining positive for IgG4, onion-skin pattern fibrosis, and lobular atrophy in the medium sized pancreatic duct that was consistent with AIP.
Conclusion: IgG4 related sclerosing disease is a systemic disorder that may involve multiple organs. The clinical manifestations are similar to that of pancreatic malignancy. For this reason, diagnostic evaluation to differentiate the two disease entities is crucial, as treatments differ significantly. Treatment for AIP consists of early initiation with glucocorticoid therapy. However, poor response to treatment usually indicates advanced fibrotic changes or an alternative diagnosis. Failure to differentiate autoimmune pancreatitis from pancreatic cancer can lead to unnecessary surgery of the pancreas along with complications associated with surgery.

Keywords: Autoimmune pancreatitis, IgG4 related sclerosing disease, Pancreatic cancer


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Author Contributions
Ashwad Afzal – Substantial contribution to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Seema Chittalae – Substantial contribution to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ivan Wong – Substantial contribution to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published.
Petros Efthimiou – Substantial contribution to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published.
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Ashwad Afzald 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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