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Republished: Recent advances in autoimmune pancreatitis: type 1 and type 2
  1. Terumi Kamisawa1,
  2. Suresh T Chari2,
  3. Markus M Lerch3,
  4. Myung-Hwan Kim4,
  5. Thomas M Gress5,
  6. Tooru Shimosegawa6
  1. 1Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
  2. 2Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Medicine A, University Medicine Greifswald, Greifswald, Germany
  4. 4Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
  5. 5Department of Gastroenterology, Endocrinology, Metabolism and Infectiology, Philipps-Universität Marburg, Marburg, Germany
  6. 6Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
  1. Correspondence to Dr Terumi Kamisawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan; kamisawa{at}cick.jp

Abstract

Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multiorgan disease, recently named IgG4-related disease. Little is known about the pathogenesis of either form of AIP. Despite frequent association of type 1 AIP with elevated serum IgG4 levels and infiltration with IgG4-positive plasma cells, it is unlikely that IgG4 plays a pathogenic role in AIP. Type 1 AIP responds to steroids, but there needs to be consensus on treatment regimens for induction and therapeutic end points. Relapses are common, but can be reduced by long-term use of low-dose steroids. Recent reports suggest that immunomodulators (azathioprine, 6-mercaptopurine and mycophenolate mofetil), as well biological agents (the antibody to CD20, rituximab) may have a role in maintaining remission in relapsing type 1 AIP. Future studies should clarify the best management options for treatment of relapses and maintenance of remission. Type 2 AIP is a pancreas-specific disorder not associated with IgG4. It presents in younger individuals equally with obstructive jaundice and pancreatitis. The inflammatory process responds to steroid therapy; relapses are uncommon. The clinical spectrum and long-term outcomes of medically treated type 2 AIP are still being evaluated.

  • Pancreatitis

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