Current Gastroenterology Reports

, Volume 14, Issue 2, pp 95–105

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

Pancreas (CE Forsmark, Section Editor)

Abstract

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.

Keywords

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

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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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