title: Recent developments in steroid-responsive pancreatitides (autoimmune pancreatitis).
abstract: OBJECTIVE
  There are two distinct steroid responsive chronic fibro-inflammatory diseases of the pancreas, called type 1 and type 2 autoimmune pancreatitis (AIP). We review recent progress in this field.
  
  
  RESULTS
  It has recently been suggested that the term AIP be used to describe type 1 AIP and the term idiopathic duct-centric chronic pancreatitis (IDCP) be used for type 2 AIP. Clinical features and long-term outcomes of AIP and IDCP are well characterized and prognosis of both diseases is excellent. Diagnostic strategies tailored to regional practice patterns have emerged with the application of International Consensus Diagnostic Criteria for AIP. Although corticosteroids remain the mainstay of treatment, management of relapses and strategies for preventing multiple relapses are better understood, including the role of maintenance therapy and B-cell depletion therapy with rituximab. Association studies with malignancies have yielded conflicting results regarding risk of cancer in AIP.
  
  
  CONCLUSIONS
  The treatment, follow-up guidelines and associations continue to evolve with our increasing experience with both AIP and IDCP. In AIP, rituximab can be used for both induction and maintenance of remission. IDCP responds to steroids without need for maintenance therapy. Both AIP and IDCP have excellent prognosis.
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, 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 / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007113:Immunity, Innate / Q000187:drug effects*; D007155:Immunologic Factors / Q000627:therapeutic use*; D010179:Pancreas / Q000473:pathology*; D050500:Pancreatitis, Chronic / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D017410:Practice Guidelines as Topic; D011379:Prognosis; D000069283:Rituximab / Q000627:therapeutic use*; D055502:Secondary Prevention
chemical_list: D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab