Journal of Gastroenterology

, Volume 45, Issue 5, pp 471–477 | Cite as

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

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 pancreatitis Steroid therapy IgG4 

References

  1. 1.
    Wakabayashi T, Kawaura Y, Satomura Y, Watanabe H, Motoo Y, Sawabu N. Long-term prognosis of duct-narrowing chronic pancreatitis. Strategy for steroid treatment. Pancreas. 2005;30:31–9.PubMedGoogle Scholar
  2. 2.
    Kamisawa T, Yoshiike M, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology. 2005;5:234–40.CrossRefPubMedGoogle Scholar
  3. 3.
    Kubota K, Iida H, Fujisawa T, Yoneda M, Inamori M, Abe Y, et al. Clinical factors predictive of spontaneous remission or relapse in cases of autoimmune pancreatitis. Gastrointest Endosc. 2007;66:1142–51.CrossRefPubMedGoogle Scholar
  4. 4.
    Ozden I, Dizdaroglu F, Poyanli A, Emre A. Spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis. Pancreatology. 2005;5:300–3.CrossRefPubMedGoogle Scholar
  5. 5.
    Araki J, Tsujimoto F, Ohta T, Nakajima Y. Natural course of autoimmune pancreatitis without steroid therapy showing hypoechoic masses in the uncinate process and tail of the pancreas on ultrasonography. J Ultrasound Med. 2006;25:1063–7.PubMedGoogle Scholar
  6. 6.
    Nishimori I, Okazaki K, Kawa S, Otsuki M. Treatment for autoimmune pancreatitis. J Biliary Tract Pancreas (in Japanese). 2007;28:961–6.Google Scholar
  7. 7.
    Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41:613–25.CrossRefPubMedGoogle Scholar
  8. 8.
    Nishimori I, Okazaki K, Suda K, Kawa S, Kamisawa T, Tanaka S, et al. Treatment for autoimmune pancreatitis. Consensus of treatment for autoimmune pancreatitis by the Research Committee of Intractable Pancreatic Diseases supported by Ministry of Health, Labour and Welfare of Japan. Suizou (in Japanese). 2005;20:343–8.Google Scholar
  9. 9.
    Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.CrossRefPubMedGoogle Scholar
  10. 10.
    Kamisawa T, Okamoto A, Wakabayashi T, Watanabe H, Sawabu N. Appropriate steroid therapy for autoimmune pancreatitis based on long-term outcome. Scand J Gastroenterl. 2008;43:609–13.CrossRefGoogle Scholar
  11. 11.
    Kamisawa T, Egawa N, Inokuma S, Tsuruta K, Okamoto A, Kamata N, et al. Pancreatic endocrine and exocrine function and salivary gland function in autoimmune pancreatitis before and after steroid therapy. Pancreas. 2003;27:235–8.CrossRefPubMedGoogle Scholar
  12. 12.
    Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626–31.CrossRefPubMedGoogle Scholar
  13. 13.
    Pearson RK, Longnecker DS, Chari ST, Smyrk TC, Okazaki K, Frulloni L, et al. Controversies in clinical pancreatology. Autoimmune pancreatitis: does it exist? Pancreas. 2003;27:1–13.CrossRefPubMedGoogle Scholar
  14. 14.
    Ghazale A, Chari ST. Optimising corticosteroid treatment for autoimmune pancreatitis. Gut. 2007;56:1650–2.CrossRefPubMedGoogle Scholar
  15. 15.
    Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006;355:2670–6.CrossRefPubMedGoogle Scholar
  16. 16.
    Park DH, Kim MH, Oh HB, Kwon OJ, Choi YJ, Lee SS, et al. Substitution of aspartic acid at position 57 of the DQβ1 affects relapse of autoimmune pancreatitis. Gastroenterology. 2008;134:440–6.CrossRefGoogle Scholar
  17. 17.
    Matsushita M, Yamashina M, Ikeura T, Shimatani M, Uchida K, Takaoka M, et al. Effective steroid pulse therapy for the biliary stenosis caused by autoimmune pancreatitis. Am J Gastroenterol. 2007;102:220–1.CrossRefPubMedGoogle Scholar
  18. 18.
    Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Morphological changes after steroid therapy in autoimmune pancreatitis. Scand J Gastroenterol. 2004;11:1154–8.CrossRefGoogle Scholar
  19. 19.
    Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, et al. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut. 2007;56:1719–24.CrossRefPubMedGoogle Scholar
  20. 20.
    Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.PubMedGoogle Scholar
  21. 21.
    Nishimori I, Otsuki M. Study about steroid therapy for autoimmune pancreatitis. Annual reports of Research Committee of Intractable Pancreatic Diseases supported by Ministry of Health, Labour and Welfare of Japan (in Japanese). 2008;137–44.Google Scholar
  22. 22.
    Nishino T, Toki F, Oyama H, Shimizu K, Shiratori K. Long-term outcome of autoimmune pancreatitis after oral prednisolone therapy. Intern Med. 2006;45:497–501.CrossRefPubMedGoogle Scholar
  23. 23.
    Okazaki K, Nichimori I, Uchida K, Otsuki M. Study about indication of therapy and relapse in autoimmune pancreatitis—therapeutic effect and evaluation method of disease activity. Annual reports of the Research Committee of Intractable Pancreatic Diseases supported by Ministry of Health, Labour and Welfare of Japan (in Japanese). 2008;133–6.Google Scholar
  24. 24.
    Nishimori I, Tamakoshi A, Kawa S, Tanaka S, Takeuchi K, Kamisawa T, et al. Influence of steroid therapy on the course of diabetes mellitus in patients with autoimmune pancreatitis: findings from a nationwide survey in Japan. Pancreas. 2006;32:244–8.CrossRefPubMedGoogle Scholar
  25. 25.
    Ito T, Nishimori I, Inoue N, Kawabe K, Gibo J, Arita Y, et al. Treatment for autoimmune pancreatitis: consensus on the treatment for patients with autoimmune pancreatitis in Japan. J Gastroenterol. 2007;42(Suppl 18):50–8.CrossRefPubMedGoogle Scholar
  26. 26.
    Ito T, Kawabe K, Arita Y, Hisano T, Igarashi H, Funakoshi A, et al. Evaluation of pancreatic endocrine and exocrine function in patients with autoimmune pancreatitis. Pancreas. 2007;34:254–9.CrossRefPubMedGoogle Scholar
  27. 27.
    Tanaka S, Kobayashi T, Nakanishi K, Okubo M, Murase T, Hashimoto M, et al. Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis. Lancet. 2000;356:910–1.CrossRefPubMedGoogle Scholar
  28. 28.
    Weber SM, Cubukcu-Dimopulo O, Palesty JA, Suriawinata A, Klimstra D, Brennan MF, et al. Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinoma. J Gastrointest Surg. 2003;7:129–37.CrossRefPubMedGoogle Scholar
  29. 29.
    Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, Abraham SC, Yeo CJ, Lillemoe KD, et al. Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg. 2003;237:853–9.CrossRefPubMedGoogle Scholar
  30. 30.
    Schnelldorfer T, Lewin DN, Adams DB. Long-term results after surgery for autoimmune sclerosing pancreatitis. J Gastrointest Surg. 2007;11:56–8.CrossRefPubMedGoogle Scholar
  31. 31.
    Lowenfels AB, Maisonneuve P, Cavallini G, Ammann RW, Lankisch PG, Andersen JR, et al. Pancreatitis and the risk of pancreatic cancer. N Engl J Med. 1993;328:1422–7.CrossRefGoogle Scholar
  32. 32.
    Takayama M, Hamano H, Ochi Y, Saegusa H, Komatsu K, Muraki T, et al. Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol. 2004;99:932–7.CrossRefPubMedGoogle Scholar
  33. 33.
    Kamisawa T, Okamoto A. Prognosis of autoimmune pancreatitis. J Gastroenterol. 2007;42(Suppl 18):59–62.CrossRefPubMedGoogle Scholar
  34. 34.
    Inoue H, Miyatani H, Sawada Y, Yoshida Y. A case of pancreatic cancer with autoimmune pancreatitis. Pancreas. 2006;33:208–9.CrossRefPubMedGoogle Scholar
  35. 35.
    Fukui T, Mitsuya T, Takaoka M, Uchida K, Matsushita M, Okazaki K. Pancreatic cancer associated with autoimmune pancreatitis in remission. Intern Med. 2008;47:151–5.CrossRefPubMedGoogle Scholar
  36. 36.
    Ghazale A, Chari S. Is autoimmune pancreatitis a risk factor for pancreatic cancer? Pancreas. 2007;35:376.CrossRefPubMedGoogle Scholar
  37. 37.
    Witkiewicz AK, Kennedy EP, Kennedy L, Yeo CJ, Hruban RH. Synchronous autoimmune pancreatitis and infiltrating pancreatic ductal adenocarcinoma: case report and review of the literature. Hum Pathol. 2008;39:1548–51.CrossRefPubMedGoogle Scholar
  38. 38.
    Sakashita F, Tanahashi T, Yamaguchi K, Osada S, Sugiyama Y, Adachi Y. Case of pancreatic tail cancer associated with autoimmune pancreatitis. Jpn J Gastroenterol Surg. 2006;39:78–83.Google Scholar
  39. 39.
    Iida H, Kubota K, Mawatari H, Yoneda M, Goto A, Abe Y, et al. A case of autoimmune pancreatitis developed pancreatic tail cancer. Suizou. 2008;23:608–14.CrossRefGoogle Scholar
  40. 40.
    Kamisawa T, Tsuruta K, Okamoto A, Horiguchi S, Hayashi Y, Xun X, et al. Frequent and significant K-ras mutation in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis. Pancreas. 2009;38:890–5.CrossRefPubMedGoogle Scholar

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

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