Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 III. Treatment and prognosis of autoimmune pancreatitis

Terumi Kamisawa, Kazuichi Okazaki, Shigeyuki Kawa, Tetsuhide Ito, Kazuo Inui, Hiroyuki Irie, Takayoshi Nishino, Kenji Notohara, Isao Nishimori, Shigeki Tanaka, Toshimasa Nishiyama, Koichi Suda, Keiko Shiratori, Masao Tanaka, Tooru Shimosegawa, Keishi Kubo, Hirotaka Ohara, Atsushi Irisawa, Yasunari Fujinaga, Osamu HasebeHiroshi Yamamoto, Kazushige Uchida, Atsushi Kanno, Kensuke Kubota, Shigeru Ko, Junichi Sakagami, Kyoko Shimizu, Masanori Sugiyama, Minoru Tada, Takahiro Nakazawa, Hirokazu Nishino, Hideaki Hamano, Yoshiki Hirooka, Kenji Hirano, Atsushi Masamune, Atsuhiro Masuda, Nobumasa Mizuno, Koji Yamaguchi, Hitoshi Yoshida

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181 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)961-970
Number of pages10
JournalJournal of Gastroenterology
Issue number6
Publication statusPublished - 06-2014
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Gastroenterology


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