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Gastrointest Endosc Clin N Am. 2018 Oct;28(4):493-519. doi: 10.1016/j.giec.2018.05.002. Epub 2018 Aug 1.

Management of Autoimmune Pancreatitis.

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Department of Medicine, Yale School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA; Department of Medicine, Waterbury Internal Medicine Residency Program, Waterbury Hospital, Yale New Haven Hospital, Main 3, 64 Robbins Street, Waterbury, CT 06708, USA.
Section of Digestive Diseases, Yale University School of Medicine, Yale Center for Pancreatic Disease, Yale University, LMP 1080, 15 York Street, New Haven, CT 06510, USA. Electronic address:


Type 1 autoimmune pancreatitis (AIP) is an IgG-4-related systemic disease that can manifest as a pancreatic disorder or another disorder of presumed autoimmune origin. Type 2 disease is typically characterized by absent IgG-4-positive cells. As patients often present with acute pancreatitis, obstructive jaundice, or pancreatic mass, it is imperative to exclude malignancy, a more common diagnosis. AIP may respond to corticosteroids, and has a strong association with other immune-mediated diseases. Recent literature suggests the benefit of immune-modulating therapy, including rituximab, although no consensus exists. This review covers the essentials of diagnosis, but focuses primarily on management of AIP.


Autoimmune pancreatitis; Azathioprine; Corticosteroids; Idiopathic duct centric pancreatitis; IgG-4; Immunomodulator; Lymphoplasmacytic sclerosing pancreatitis; Rituximab

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