Recent advances in autoimmune pancreatitis: Type 1 and type 2

Terumi Kamisawa, Suresh T. Chari, Markus M. Lerch, Myung Hwan Kim, Thomas M. Gress, Tooru Shimosegawa

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109 Scopus citations

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 longterm outcomes of medically treated type 2 AIP are still being evaluated.

Original languageEnglish (US)
Pages (from-to)1373-1380
Number of pages8
JournalGut
Volume62
Issue number9
DOIs
StatePublished - Sep 1 2013

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ASJC Scopus subject areas

  • Gastroenterology

Cite this

Kamisawa, T., Chari, S. T., Lerch, M. M., Kim, M. H., Gress, T. M., & Shimosegawa, T. (2013). Recent advances in autoimmune pancreatitis: Type 1 and type 2. Gut, 62(9), 1373-1380. https://doi.org/10.1136/gutjnl-2012-304224