title: Autoimmune pancreatitis: an update on classification, diagnosis, natural history and management.
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.
authors.affiliation: Fiterman Center for Digestive Diseases, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Raghuwansh P
authors.initials: RP
authors.lastname: Sah
authors.forename: Suresh T
authors.initials: ST
authors.lastname: Chari
mesh_terms: D001327:Autoimmune Diseases / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D010195:Pancreatitis / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D011379:Prognosis; D012008:Recurrence