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

Curr Gastroenterol Rep. 2012 Apr;14(2):95-105. doi: 10.1007/s11894-012-0246-8.


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.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases* / classification
  • Autoimmune Diseases* / diagnosis
  • Autoimmune Diseases* / etiology
  • Autoimmune Diseases* / therapy
  • Humans
  • Pancreatitis* / classification
  • Pancreatitis* / diagnosis
  • Pancreatitis* / etiology
  • Pancreatitis* / therapy
  • Prognosis
  • Recurrence