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

  • Pancreas (CE Forsmark, Section Editor)
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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.

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Sah, R.P., Chari, S.T. Autoimmune Pancreatitis: An Update on Classification, Diagnosis, Natural History and Management. Curr Gastroenterol Rep 14, 95–105 (2012). https://doi.org/10.1007/s11894-012-0246-8

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