Skip to main content

Abstract

Autoimmune pancreatitis (AIP) is a chronic, fibro-inflammatory disease of the pancreas that has been recognized as a distinct clinical entity for approximately two decades. Since patients with this rare disorder commonly present with obstructive jaundice mimicking pancreatic cancer, early recognition and diagnosis of AIP are critical. Over the past few years, AIP has been shown to be comprised of two distinct subtypes—type 1 and type 2 AIP. Type 1 AIP is the pancreatic manifestation of a systemic disorder known as IgG4-related disease, and is commonly associated with extrapancreatic manifestations such as sclerosing cholangitis. On the other hand, type 2 AIP is a pancreatic disorder without extrapancreatic manifestations that is not related to IgG4, and typically occurs in younger patients. This chapter provides a review of current knowledge of autoimmune pancreatitis with a focus on biliary manifestations of the disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Yoshida K, Toki F, Takeuchi T, Watanabe S-I, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Dig Dis Sci. 1995;40(7):1561–8.

    Article  PubMed  CAS  Google Scholar 

  2. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51.

    Article  PubMed  CAS  Google Scholar 

  3. Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Recent advances in autoimmune pancreatitis: type 1 and type 2. Gut. 2013;62(9):1373–80.

    Article  PubMed  CAS  Google Scholar 

  4. Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas—an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–98.

    Article  PubMed  CAS  Google Scholar 

  5. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732–8.

    Article  PubMed  CAS  Google Scholar 

  6. Kamisawa T. IgG4-positive plasma cells specifically infiltrate various organs in autoimmune pancreatitis. Pancreas. 2004;29(2):167–8.

    Article  PubMed  Google Scholar 

  7. Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003;27(8):1119–27.

    Article  PubMed  Google Scholar 

  8. Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40(3):352–8.

    Article  PubMed  Google Scholar 

  9. Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T, et al. Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39(5):549–54.

    Article  PubMed  CAS  Google Scholar 

  10. Sah RP, Chari ST. Autoimmune pancreatitis: an update on classification, diagnosis, natural history and management. Curr Gastroenterol Rep. 2012;14(2):95–105.

    Article  PubMed  Google Scholar 

  11. Nishimori I, Tamakoshi A, Otsuki M, Research Committee on Intractable Diseases of the Pancreas, Ministry of Health, Labour, and Welfare of Japan. Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol. 2007;42 Suppl 18:6–8.

    Article  PubMed  Google Scholar 

  12. Kamisawa T, Chari ST, Giday SA, Kim MH, Chung JB, Lee KT, et al. Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas. 2011;40(6):809–14.

    Article  PubMed  CAS  Google Scholar 

  13. Sugumar A, Kloppel G, Chari ST. Autoimmune pancreatitis: pathologic subtypes and their implications for its diagnosis. Am J Gastroenterol. 2009;104(9):2308–10. quiz 2311.

    Article  PubMed  Google Scholar 

  14. Kojima M, Sipos B, Klapper W, Frahm O, Knuth HC, Yanagisawa A, et al. Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol. 2007;31(4):521–8.

    Article  PubMed  Google Scholar 

  15. Hart PA, KamiSawa T, Brugge WR, Chung JB, Culver EL, Czako L. Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut. 2012;62:1771–6.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, et al. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol. 2009;104(9):2295–306.

    Article  PubMed  Google Scholar 

  17. 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(5):932–7.

    Article  PubMed  Google Scholar 

  18. Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology. 2010;139(1):140–8. quiz e112-143.

    Article  PubMed  Google Scholar 

  19. Ohara H, Okazaki K, Tsubouchi H, Inui K, Kawa S, Kamisawa T, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 2012;19(5):536–42.

    Article  PubMed  Google Scholar 

  20. Okazaki K, Uchida K, Koyabu M, Miyoshi H, Takaoka M. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol. 2011;46(3):277–88.

    Article  PubMed  CAS  Google Scholar 

  21. Okazaki K, Uchida K, Miyoshi H, Ikeura T, Takaoka M, Nishio A. Recent concepts of autoimmune pancreatitis and IgG4-related disease. Clin Rev Allergy Immunol. 2011;41(2):126–38.

    Article  PubMed  CAS  Google Scholar 

  22. Hirano K, Tada M, Isayama H, Yamamoto K, Mizuno S, Yagioka H, et al. Endoscopic evaluation of factors contributing to intrapancreatic biliary stricture in autoimmune pancreatitis. Gastrointest Endosc. 2010;71(1):85–90.

    Article  PubMed  Google Scholar 

  23. Nakazawa T, Ohara H, Sano H, Aoki S, Kobayashi S, Okamoto T, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc. 2004;60(6):937–44.

    Article  PubMed  Google Scholar 

  24. Okazaki K, Uchida K, Koyabu M, Miyoshi H, Ikeura T, Takaoka M. IgG4 cholangiopathy—current concept, diagnosis, and pathogenesis. J Hepatol. 2014;61(3):690–5.

    Article  PubMed  CAS  Google Scholar 

  25. Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas. 2006;32(2):229.

    Article  PubMed  Google Scholar 

  26. Takikawa H, Takamori Y, Tanaka A, Kurihara H, Nakanuma Y. Analysis of 388 cases of primary sclerosing cholangitis in Japan; Presence of a subgroup without pancreatic involvement in older patients. Hepatol Res. 2004;29(3):153–9.

    Article  PubMed  Google Scholar 

  27. Naitoh I, Nakazawa T, Ohara H, Ando T, Hayashi K, Tanaka H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis. J Gastroenterol. 2009;44(11):1147–55.

    Article  PubMed  Google Scholar 

  28. Tomiyama T, Uchida K, Matsushita M, Ikeura T, Fukui T, Takaoka M, et al. Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis. J Gastroenterol. 2011;46(5):696–704.

    Article  PubMed  CAS  Google Scholar 

  29. Nakazawa T, Ohara H, Sano H, Ando T, Aoki S, Kobayashi S, et al. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas. 2005;30(1):20–5.

    PubMed  Google Scholar 

  30. 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(3):706–15.

    Article  PubMed  Google Scholar 

  31. Proctor RD, Rofe CJ, Bryant TJ, Hacking CN, Stedman B. Autoimmune pancreatitis: an illustrated guide to diagnosis. Clin Radiol. 2013;68(4):422–32.

    Article  PubMed  CAS  Google Scholar 

  32. Takahashi N, Fletcher JG, Hough DM, Fidler JL, Kawashima A, Mandrekar JN, et al. Autoimmune pancreatitis: differentiation from pancreatic carcinoma and normal pancreas on the basis of enhancement characteristics at dual-phase CT. AJR Am J Roentgenol. 2009;193(2):479–84.

    Article  PubMed  Google Scholar 

  33. Kwak SW, Kim S, Lee JW, Lee NK, Kim CW, Yi MS, et al. Evaluation of unusual causes of pancreatitis: role of cross-sectional imaging. Eur J Radiol. 2009;71(2):296–312.

    Article  PubMed  Google Scholar 

  34. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006;355(25):2670–6.

    Article  PubMed  CAS  Google Scholar 

  35. Kamisawa T, Chen PY, Tu Y, Nakajima H, Egawa N, Tsuruta K, et al. MRCP and MRI findings in 9 patients with autoimmune pancreatitis. World J Gastroenterol. 2006;12(18):2919–22.

    PubMed  PubMed Central  Google Scholar 

  36. Park SH, Kim MH, Kim SY, Kim HJ, Moon SH, Lee SS, et al. Magnetic resonance cholangiopancreatography for the diagnostic evaluation of autoimmune pancreatitis. Pancreas. 2010;39(8):1191–8.

    Article  PubMed  Google Scholar 

  37. Kawamoto S, Siegelman SS, Hruban RH, Fishman EK. Lymphoplasmacytic sclerosing pancreatitis (autoimmune pancreatitis): evaluation with multidetector CT. Radiographics. 2008;28(1):157–70.

    Article  PubMed  Google Scholar 

  38. Moon SH, Kim MH. The role of endoscopy in the diagnosis of autoimmune pancreatitis. Gastrointest Endosc. 2012;76(3):645–56.

    Article  PubMed  Google Scholar 

  39. Hoki N, Mizuno N, Sawaki A, Tajika M, Takayama R, Shimizu Y, et al. Diagnosis of autoimmune pancreatitis using endoscopic ultrasonography. J Gastroenterol. 2009;44(2):154–9.

    Article  PubMed  Google Scholar 

  40. Park DH, Kim MH, Chari ST. Recent advances in autoimmune pancreatitis. Gut. 2009;58(12):1680–9.

    Article  PubMed  CAS  Google Scholar 

  41. Nakanuma Y, Zen Y. Pathology and immunopathology of immunoglobulin G4-related sclerosing cholangitis: The latest addition to the sclerosing cholangitis family. Hepatol Res. 2007;37 Suppl 3:S478–86.

    Article  PubMed  CAS  Google Scholar 

  42. Naitoh I, Zen Y, Nakazawa T, Ando T, Hayashi K, Okumura F, et al. Small bile duct involvement in IgG4-related sclerosing cholangitis: liver biopsy and cholangiography correlation. J Gastroenterol. 2011;46(2):269–76.

    Article  PubMed  CAS  Google Scholar 

  43. Umemura T, Zen Y, Hamano H, Kawa S, Nakanuma Y, Kiyosawa K. Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology. 2007;46(2):463–71.

    Article  PubMed  CAS  Google Scholar 

  44. Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol. 2007;102(8):1646–53.

    Article  PubMed  CAS  Google Scholar 

  45. Morselli-Labate AM, Pezzilli R. Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: a systematic literature review and meta-analysis. J Gastroenterol Hepatol. 2009;24(1):15–36.

    Article  PubMed  Google Scholar 

  46. Sah RP, Chari ST. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011;23(1):108–13.

    Article  PubMed  CAS  Google Scholar 

  47. Umemura T, Ota M, Hamano H, Katsuyama Y, Muraki T, Arakura N, et al. Association of autoimmune pancreatitis with cytotoxic T-lymphocyte antigen 4 gene polymorphisms in Japanese patients. Am J Gastroenterol. 2008;103(3):588–94.

    Article  PubMed  CAS  Google Scholar 

  48. Schwaiger T, van den Brandt C, Fitzner B, Zaatreh S, Kraatz F, Dummer A, et al. Autoimmune pancreatitis in MRL/Mp mice is a T cell-mediated disease responsive to cyclosporine A and rapamycin treatment. Gut. 2014;63(3):494–505.

    Article  PubMed  CAS  Google Scholar 

  49. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–6.

    Article  PubMed  Google Scholar 

  50. Moon SH, Kim MH, Park DH, Hwang CY, Park SJ, Lee SS. Is a 2-week steroid trial after initial negative investigation for malignancy useful in differentiating autoimmune pancreatitis from pancreatic cancer? a propsective outcomes study. Gut. 2008;57:1704–12.

    Article  PubMed  CAS  Google Scholar 

  51. Chari ST, Takahashi N, Levy MJ, Smyrk TC, Clain JE, Pearson RK, et al. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clin Gastroenterol Hepatol. 2009;7(10):1097–103.

    Article  PubMed  CAS  Google Scholar 

  52. Sugumar A, Levy MJ, Kamisawa T, Webster GJ, Kim MH, Enders F, et al. Endoscopic retrograde pancreatography criteria to diagnose autoimmune pancreatitis: an international multicentre study. Gut. 2011;60(5):666–70.

    Article  PubMed  Google Scholar 

  53. Moon SH, Kim MH, do Park H, Song TJ, Eum J, Lee SS, et al. IgG4 immunostaining of duodenal papillary biopsy specimens may be useful for supporting a diagnosis of autoimmune pancreatitis. Gastrointest Endosc. 2010;71(6):960–6.

    Article  PubMed  Google Scholar 

  54. Mizuno N, Bhatia V, Hosoda W, Sawaki A, Hoki N, Hara K, et al. Histological diagnosis of autoimmune pancreatitis using EUS-guided trucut biopsy: a comparison study with EUS-FNA. J Gastroenterol. 2009;44(7):742–50.

    Article  PubMed  CAS  Google Scholar 

  55. Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504–7.

    Article  PubMed  CAS  Google Scholar 

  56. Kim MH, Moon SH. The need for a consensus on the definition of remission of autoimmune pancreatitis after steroid treatment. Gut. 2010;59(12):1730.

    Article  PubMed  Google Scholar 

  57. Pannala R, Chari ST. Corticosteroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1438–9.

    Article  PubMed  CAS  Google Scholar 

  58. Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Pancreas. 2013;62:1607–15.

    CAS  Google Scholar 

  59. Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2008;6(3):364–6.

    Article  PubMed  CAS  Google Scholar 

  60. Kamisawa T, Okazaki K, Kawa S, Shimosegawa T, Tanaka M, Research Committee for Intractable Pancreatic D, et al. Japanese consensus guidelines for management of autoimmune pancreatitis: III. Treatment and prognosis of AIP. J Gastroenterol. 2010;45(5):471–7.

    Article  PubMed  CAS  Google Scholar 

  61. Frulloni L, Scattolini C, Falconi M, Zamboni G, Capelli P, Manfredi R, et al. Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol. 2009;104(9):2288–94.

    Article  PubMed  Google Scholar 

  62. 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(6):1142–51.

    Article  PubMed  Google Scholar 

  63. Shiokawa M, Kodama Y, Yoshimura K, Kawanami C, Mimura J, Yamashita Y, et al. Risk of cancer in patients with autoimmune pancreatitis. Am J Gastroenterol. 2013;108(4):610–7.

    Article  PubMed  CAS  Google Scholar 

  64. Hart PA, Law RJ, Dierkhising RA, Smyrk TC, Takahashi N, Chari ST. Risk of cancer in autoimmune pancreatitis: a case–control study and review of the literature. Pancreas. 2014;43(3):417–21.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Sunil Sheth M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Sengupta, N., Sheth, S. (2015). Autoimmune Pancreatitis. In: Dixon, E., Vollmer Jr., C., May, G. (eds) Management of Benign Biliary Stenosis and Injury. Springer, Cham. https://doi.org/10.1007/978-3-319-22273-8_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-22273-8_6

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22272-1

  • Online ISBN: 978-3-319-22273-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics