Advertisement

Approach to Therapy

Chapter

Abstract

Autoimmune pancreatitis is a distinct form of chronic pancreatitis that is characterized by a dramatic response to steroid treatment. In addition to resolution of the pancreatic manifestations, disease activity in other affected organs is also steroid responsive. The primary goal of treatment is reduction in acute inflammation and hopefully to prevent progression to irreversible fibrosis. Initial treatment also often requires management of diabetes mellitus and biliary obstruction. Though initial response to steroids is invariable, relapses are common. Nearly 50 % of patients relapse after initial therapy if no maintenance treatment is given, particularly those with involvement of the proximal biliary tract. Disease relapses generally occur in the pancreas or biliary tree; however, they can also develop in other previously unaffected organs. The remission rates using steroid treatment for disease relapses remain very high. There are several options for preventing relapses including long-term low-dose steroids, steroid-sparing immunomodulators, and rituximab; however, data supporting these strategies is just emerging.

Keywords

Serum IgG4 Steroid Treatment Obstructive Jaundice Mycophenolate Mofetil Disease Relapse 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Sumida T, Mimori T, Tanaka Y, Tsubota K, Yoshino T, Kawa S, Suzuki R, Takegami T, Tomosugi N, Kurose N, Ishigaki Y, Azumi A, Kojima M, Nakamura S, Inoue D. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22:1–14.Google Scholar
  2. 2.
    Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–6. quiz 934.Google Scholar
  3. 3.
    Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Eng J Med. 2001;344:732–8.CrossRefGoogle Scholar
  4. 4.
    Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, Nakai Y, Sasahira N, Tsujino T, Yoshida H, Kawabe T, Omata M. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut. 2007;56:1719–24.PubMedCrossRefGoogle Scholar
  5. 5.
    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;5:234–8. discussion 238–40.PubMedCrossRefGoogle Scholar
  6. 6.
    Nishino T, Toki F, Oyama H, Oi I, Kobayashi M, Takasaki K, Shiratori K. Biliary tract involvement in autoimmune pancreatitis. Pancreas. 2005;30:76–82.PubMedGoogle Scholar
  7. 7.
    Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Morphological changes after steroid therapy in autoimmune pancreatitis. Scand J Gastroenterol. 2004;39:1154–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Moon SH, Kim MH, Park DH, Hwang CY, Park SJ, Lee SS, Seo DW, Lee SK. Is a 2-week steroid trial after initial negative investigation for malignancy useful in differentiating autoimmune pancreatitis from pancreatic cancer? A prospective outcome study. Gut. 2008;57:1704–12.PubMedCrossRefGoogle Scholar
  9. 9.
    Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, Okumura F, Nishikawa T, Kobayashi K, Ichiya T, Takatori H, Yamakita K, Kubota K, Hamano H, Okamura K, Hirano K, Ito T, Ko SB, Omata M. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Ryu JK, Chung JB, Park SW, Lee JK, Lee KT, Lee WJ, Moon JH, Cho KB, Kang DW, Hwang JH, Yoo KS, Yoo BM, Lee DH, Kim HK, Moon YS, Lee J, Lee HS, Choi HS, Lee SK, Kim YT, Kim CD, Kim SJ, Hahm JS, Yoon YB. Review of 67 patients with autoimmune pancreatitis in Korea: a multicenter nationwide study. Pancreas. 2008;37:377–85.PubMedCrossRefGoogle Scholar
  11. 11.
    Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, Pearson RK, Smyrk TC, Petersen BT, Topazian MD, Takahashi N, Farnell MB, Vege SS. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology. 2010;139:140–8. quiz e12–3.PubMedCrossRefGoogle Scholar
  12. 12.
    Sandanayake NS, Church NI, Chapman MH, Johnson GJ, Dhar DK, Amin Z, Deheragoda MG, Novelli M, Winstanley A, Rodriguez-Justo M, Hatfield AR, Pereira SP, Webster GJ. Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2009;7:1089–96.PubMedCrossRefGoogle Scholar
  13. 13.
    Zamboni G, Luttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Kloppel G. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445:552–63.PubMedCrossRefGoogle Scholar
  14. 14.
    Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, Topazian MD, Clain JE, Pearson RK, Petersen BT, Vege SS, Lindor K, Farnell MB. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.PubMedCrossRefGoogle Scholar
  15. 15.
    Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol. 2009;104:2295–306.PubMedCrossRefGoogle Scholar
  16. 16.
    Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, Chari ST. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2008;6:364–6.PubMedCrossRefGoogle Scholar
  17. 17.
    Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine. 2012;91:57–66.PubMedCrossRefGoogle Scholar
  18. 18.
    Su C, Lichtenstein GR. Treatment of inflammatory bowel disease with azathioprine and 6-mercaptopurine. Gastroenterol Clin North Am. 2004;33:209–34, viii.Google Scholar
  19. 19.
    Wang K, Zhang H, Li Y, Wei Q, Li H, Yang Y, Lu Y. Safety of mycophenolate mofetil versus azathioprine in renal transplantation: a systematic review. Transplant Proc. 2004;36:2068–70.PubMedCrossRefGoogle Scholar
  20. 20.
    Kimby E. Tolerability and safety of rituximab (MabThera). Cancer Treat Rev. 2005;31:456–73.PubMedCrossRefGoogle Scholar
  21. 21.
    Ram R, Ben-Bassat I, Shpilberg O, Polliack A, Raanani P. The late adverse events of rituximab therapy–rare but there! Leuk Lymphoma. 2009;50:1083–95.PubMedCrossRefGoogle Scholar
  22. 22.
    Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim MH, Kloppel G, Lerch MM, Lohr M, Notohara K, Okazaki K, Schneider A, Zhang L. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–8.PubMedCrossRefGoogle Scholar
  23. 23.
    Deshpande V, Gupta R, Sainani N, Sahani DV, Virk R, Ferrone C, Khosroshahi A, Stone JH, Lauwers GY. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011;35:26–35.PubMedCrossRefGoogle Scholar
  24. 24.
    Kamisawa T, Chari ST, Giday SA, Kim MH, Chung JB, Lee KT, Werner J, Bergmann F, Lerch MM, Mayerle J, Pickartz T, Lohr M, Schneider A, Frulloni L, Webster GJ, Reddy DN, Liao WC, Wang HP, Okazaki K, Shimosegawa T, Kloeppel G, Go VL. Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas. 2011;40:809–14.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Division of Gastroenterology and Hepatology, Internal MedicineMayo Clinic College of MedicineRochesterUSA

Personalised recommendations