Review
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 28, 2024; 30(8): 817-832
Published online Feb 28, 2024. doi: 10.3748/wjg.v30.i8.817
Autoimmune pancreatitis: Cornerstones and future perspectives
Camilla Gallo, Giulia Dispinzieri, Nicola Zucchini, Pietro Invernizzi, Sara Massironi
Camilla Gallo, Giulia Dispinzieri, Pietro Invernizzi, Sara Massironi, Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, Fondazione IRCCS San Gerardo dei Tintori; University of Milano-Bicocca, Monza 20900, Italy
Nicola Zucchini, Department of Pathology, Fondazione IRCCS San Gerardo dei Tintori, Monza 20900, Italy
Author contributions: Gallo C writing and supervising; Dispinzieri G writing; Zucchini N writing and figures editing; Massironi S coordinationg and supervising; Invernizzi P supervising.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Sara Massironi, MD, PhD, Chief Physician, Doctor, Medical Assistant, Research Scientist, Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, Fondazione IRCCS San Gerardo dei Tintori; University of Milano-Bicocca, 33 Via Pergolesi, Monza 20900, Italy. sara.massironi@libero.it
Received: November 15, 2023
Peer-review started: November 15, 2023
First decision: December 15, 2023
Revised: December 18, 2023
Accepted: January 25, 2024
Article in press: January 25, 2024
Published online: February 28, 2024
Core Tip

Core Tip: Autoimmune pancreatitis (AIP) is rare and often misdiagnosed. The lymphoplasmacytic sclerosing form, type 1 AIP (AIP-1), represents the pancreatic manifestation of immunoglobulin G4-related disease, while the idiopathic ductal centric form, type 2 AIP (AIP-2), is often associated with inflammatory bowel disease. AIP-1 presents with obstructive jaundice or abnormalities in exocrine and endocrine pancreatic function; AIP-2 usually shows abdominal pain and acute pancreatitis. The atypical mass-forming abnormality of the pancreas implies the need to histologically distinguish AIP form pancreatic ductal adenocarcinoma. Steroids are the first-line therapy for both AIP-1 and AIP-2, rituximab is a good alternative for AIP-1. Given the high relapse rate, long-term maintenance therapy is recommended. Scientific efforts are focusing on target therapies.