Autoimmune pancreatitis: An update on classification, diagnosis, natural history and management

Raghuwansh P. Sah, Suresh T. Chari

Research output: Contribution to journalArticlepeer-review

61 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)95-105
Number of pages11
JournalCurrent gastroenterology reports
Issue number2
StatePublished - Apr 2012


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

ASJC Scopus subject areas

  • Gastroenterology


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