title: Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 III. Treatment and prognosis of autoimmune pancreatitis.
abstract: The standard treatment for autoimmune pancreatitis (AIP) is steroid therapy, although some patients improve spontaneously. Indications for steroid therapy in AIP patients are symptoms such as obstructive jaundice, abdominal pain, back pain, and the presence of symptomatic extrapancreatic lesions. Prior to steroid therapy, obstructive jaundice should be managed by biliary drainage, and blood glucose levels should be controlled in patients with diabetes mellitus. The recommended initial oral prednisolone dose for induction of remission is 0.6 mg/kg/day, which is administered for 2-4 weeks. The dose is then tapered by 5 mg every 1-2 weeks, based on changes in clinical manifestations, biochemical blood tests (such as liver enzymes and IgG or IgG4 levels), and repeated imaging findings (US, CT, MRCP, ERCP, etc.). The dose is tapered to a maintenance dose (2.5-5 mg/day) over a period of 2-3 months. Cessation of steroid therapy should be based on the disease activity in each case. Termination of maintenance therapy should be planned within 3 years in cases with radiological and serological improvement. Re-administration or dose-up of steroid is effective for treating AIP relapse. Application of immunomodulatory drugs is considered for AIP patients who prove resistant to steroid therapy. The prognosis of AIP appears to be good over the short-term with steroid therapy. The long-term outcome is less clear, as there are many unknown factors, such as relapse, pancreatic exocrine or endocrine dysfunction, and associated malignancy.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan, kamisawa@cick.jp.
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Kawa
authors.forename: Tetsuhide
authors.initials: T
authors.lastname: Ito
authors.forename: Kazuo
authors.initials: K
authors.lastname: Inui
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Irie
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Nishino
authors.forename: Kenji
authors.initials: K
authors.lastname: Notohara
authors.forename: Isao
authors.initials: I
authors.lastname: Nishimori
authors.forename: Shigeki
authors.initials: S
authors.lastname: Tanaka
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Nishiyama
authors.forename: Koichi
authors.initials: K
authors.lastname: Suda
authors.forename: Keiko
authors.initials: K
authors.lastname: Shiratori
authors.forename: Masao
authors.initials: M
authors.lastname: Tanaka
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
mesh_terms: D044466:Asian People; D001327:Autoimmune Diseases / Q000188:drug therapy*; D032921:Consensus; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D010195:Pancreatitis / Q000188:drug therapy* / Q000276:immunology; D011379:Prognosis; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids