Current Gastroenterology Reports

, Volume 14, Issue 2, pp 95–105 | Cite as

Autoimmune Pancreatitis: An Update on Classification, Diagnosis, Natural History and Management

  • Raghuwansh P. Sah
  • Suresh T. Chari
Pancreas (CE Forsmark, Section Editor)


Autoimmune Pancreatitis (AIP) is a recently recognized chronic fibro-inflammatory disease of the pancreas. Although rare, its recognition continues to increase worldwide. Patients often present with painless obstructive jaundice mimicking pancreatic cancer. Two subtypes of AIP are known-type 1 is a multi-organ disease associated with IgG4; type 2 appears to be a pancreas-specific disorder. Dramatic response to steroid treatment is characteristic of both forms. A non-invasive diagnosis of type 1 AIP may be possible using diagnostic criteria (in ~70% cases) while diagnosis of type 2 requires histology. These subtypes differ in natural history- type 1 often relapses while initial reports suggest that type 2 does not. Long term complications include endocrine and exocrine insufficiency and in case of type 1, disease relapses and complications from extra-pancreatic involvement. Neither form affects long term survival. The treatment and follow-up guidelines continue to evolve with our increasing experience in AIP.


Autoimmune pancreatitis Chronic pancreatitis IgG4-related systemic disease Lymphoplasmacytic sclerosing pancreatitis Idiopathic duct centric pancreatitis 



No potential conflicts of interest relevant to this article were reported.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Fiterman Center for Digestive DiseasesMayo ClinicRochesterUSA
  2. 2.Miles and Shirley Fiterman Center for Digestive DiseasesMayo ClinicRochesterUSA

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