Article Text

This article has a correction. Please see:

Download PDFPDF
Recent advances in autoimmune pancreatitis
  1. D H Park1,
  2. M-H Kim1,
  3. S T Chari2
  1. 1Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
  2. 2Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr S T Chari, Miles & Shirley Fiterman Center for Digestive Diseases, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA; chari.suresh{at}


Autoimmune pancreatitis (AIP) is distinct from calcifying and obstructive forms of chronic pancreatitis. Clinically and histologically it has two distinct subsets: (i) lymphoplasmacytic sclerosing pancreatitis or type 1 AIP which appears to be a systemic disease characterised by abundant infiltration of affected organs with immunoglobulin G4 (IgG4)-positive plasma cells and (2) duct-centric or type 2 AIP characterised by granulocyte epithelial lesions in the pancreas without systemic involvement. In AIP a marked lymphoplasmacytic infiltrate that responds dramatically to steroid therapy suggests an autoimmune aetiology. However, the target autoantigen(s) and the effector cells in AIP remain speculative. Despite the consistent elevation in serum IgG4 levels and tissue infiltration with IgG4-positive plasma cells in type 1 AIP, the role of IgG4 in its pathogenesis remains unknown. Recent development of animal models of AIP will help improve our understanding of the pathogenesis of these newly described forms of chronic pancreatitis.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Funding This study (pathogenesis part of autoimmune pancreatitis) was supported by a grant from the Korea Health 21 R&D Project, Ministry of Health & Welfare, Republic of Korea (A080277).

  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Patient consent Obtained.

Linked Articles

  • Correction
    BMJ Publishing Group Ltd and British Society of Gastroenterology