Recent Advances in Autoimmune Pancreatitis.

Abstract

Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is characterized clinically by frequent presentation with obstructive jaundice, histologically by a dense lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to corticosteroid therapy. Two distinct diseases, type 1 and type 2 AIP, share these features. However, these 2 diseases have unique pancreatic histopathologic patterns and differ significantly in their demographic profiles, clinical presentation, and natural history. Recognizing the popular and long-standing association of the term "AIP" with what is now called "type 1 AIP," we suggest using "AIP" solely for type 1 AIP and to acknowledge its own distinct disease status by using "idiopathic duct-centric chronic pancreatitis" (IDCP) for type 2 AIP. AIP is the pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD). The etiopathogenesis of AIP and IgG4-RD is largely unknown. However, the remarkable effectiveness of B-cell depletion therapy with rituximab in patients with AIP and IgG4-RD highlights the crucial role of B cells in its pathogenesis. IDCP is less commonly recognized, and little is known about its pathogenesis. IDCP has no biomarker but is associated with inflammatory bowel disease in ~25% of patients. Recently, the international consensus diagnostic criteria for AIP identified combinations of features that are diagnostic of both diseases. Both AIP and IDCP are corticosteroid responsive; however, relapses are common in AIP and rare in IDCP. Therefore, maintenance therapy with either an immunomodulator (eg, azathioprine, 6-mercaptopurine, or mycophenolate mofetil) or rituximab is often necessary for patients with AIP. Long-term survival is excellent for both patients with AIP and patients with IDCP.

• Authors: Phil A Hart, Yoh Zen, Suresh T Chari
• Journal: Gastroenterology (Gastroenterology) Vol. 149 Issue 1 Pg. 39-51 (Jul 2015) ISSN: 1528-0012 [Electronic] United States
• PMID: 25770706 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2015 AGA Institute. Published by Elsevier Inc. All rights reserved.

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Immunologic Factors
• Rituximab

Topics

• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Autoimmune Diseases (drug therapy, immunology)
• B-Lymphocytes (immunology)
• Humans
• Immunologic Factors (therapeutic use)
• Pancreas (drug effects, pathology)
• Pancreatitis, Chronic (classification, drug therapy, immunology, pathology)
• Rituximab
• Treatment Outcome