Journal of Gastroenterology

, Volume 45, Issue 5, pp 471–477

Japanese consensus guidelines for management of autoimmune pancreatitis: III. Treatment and prognosis of AIP

  • Terumi Kamisawa
  • Kazuichi Okazaki
  • Shigeyuki Kawa
  • Tooru Shimosegawa
  • Masao Tanaka
  • Working members of Research Committee for Intractable Pancreatic Disease and Japan Pancreas Society
Review

DOI: 10.1007/s00535-010-0221-9

Cite this article as:
Kamisawa, T., Okazaki, K., Kawa, S. et al. J Gastroenterol (2010) 45: 471. doi:10.1007/s00535-010-0221-9

Abstract

Steroid therapy appeared to be a standard treatment for autoimmune pancreatitis (AIP), although some AIP patients improve spontaneously. The indications for steroid therapy in AIP patients are symptoms such as obstructive jaundice, abdominal pain, and back pain, and the presence of symptomatic extrapancreatic lesions. Before steroid therapy, jaundice should be managed by biliary drainage in patients with obstructive jaundice, and blood glucose levels should be controlled in patients with diabetes mellitus. For the initial oral prednisolone dose for induction of remission, 0.6 mg/kg/day is recommended. The initial dose is administered for 2–4 weeks, and the dose is tapered by 5 mg every 1–2 weeks, based on changes in the 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. Steroid therapy should be stopped based on the disease activity in each case. Stopping of maintenance therapy should be planned within at least 3 years in cases with radiological and serological improvement. Re-administration or dose-up of steroid is effective for treating AIP relapses. The prognosis of AIP appears to be good over the short-term with steroid therapy. It is unclear whether the long-term outcome is good because there are many unknown factors, such as relapse, pancreatic exocrine or endocrine dysfunction, and associated malignancy.

Keywords

Autoimmune pancreatitisSteroid therapyIgG4

Copyright information

© Springer 2010

Authors and Affiliations

  • Terumi Kamisawa
    • 1
  • Kazuichi Okazaki
    • 2
  • Shigeyuki Kawa
    • 3
  • Tooru Shimosegawa
    • 4
  • Masao Tanaka
    • 5
  • Working members of Research Committee for Intractable Pancreatic Disease and Japan Pancreas Society
  1. 1.Department of Internal MedicineTokyo Metropolitan Komagome HospitalTokyoJapan
  2. 2.Department of Gastroenterology and HepatologyKansai Medical UniversityOsakaJapan
  3. 3.Center for Health, Safety and Environmental ManagementShinshu UniversityMatsumotoJapan
  4. 4.Division of GastroenterologyTohoku University Graduate School of MedicineSendaiJapan
  5. 5.Department of Surgery and Oncology, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan