Skip to main content

Clinical Features

  • Chapter
  • First Online:
Autoimmune Pancreatitis

Abstract

Autoimmune pancreatitis (AIP) is a unique type of chronic pancreatitis that has distinct pathological, histological, and clinical characteristics [1, 2]. In 1961, Sarles et al. first described a pancreatitis associated with hypergammaglobulinemia, suggesting autoimmunity as the etiology of pancreatitis [3]. The concept of autoimmune pancreatitis (AIP) was proposed by Yoshida et al. in 1995 [4]. Since then it has become recognized as a distinct entity, and many cases have been reported in Eastern countries, as well as in Western countries [5, 6].

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

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
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. Gardner TB, Chari ST. Autoimmune pancreatitis. Gastroenterol Clin North Am. 2008;37(2):439–60.

    Google Scholar 

  2. Kim KP, Kim MH, Song MH, Lee SS, Seo DW, Lee SK. Autoimmune chronic pancreatitis. Am J Gastroenterol. 2004;99(8):1605–16.

    Article  PubMed  Google Scholar 

  3. 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  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  6. Okazaki K, Kawa S, Kamisawa T, Ito T, Inui K, Irie H, et al. Japanese clinical guidelines for autoimmune pancreatitis. Pancreas. 2009;38(8):849–66.

    Article  PubMed  Google Scholar 

  7. Maire F, Le Baleur Y, Rebours V, Vullierme MP, Couvelard A, Voitot H, et al. Outcome of patients with type 1 or 2 autoimmune pancreatitis. Am J Gastroenterol. 2011;106(1):151–6.

    Article  CAS  PubMed  Google Scholar 

  8. 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 e12–3.

    Google Scholar 

  9. Song TJ, Kim JH, Kim MH, Jang JW, Parkdo H, Lee SS, et al. Comparison of clinical findings between histologically confirmed type 1 and type 2 autoimmune pancreatitis. J Gastroenterol Hepatol. 2012;27(4):700–8.

    Article  CAS  PubMed  Google Scholar 

  10. Hart PA, Kamisawa T, Brugge WR, Chung JB, Culver EL, Czako L, et al. Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut. 2013;62(12):1771–6.

    Article  PubMed Central  PubMed  Google Scholar 

  11. Nishimori I, Tamakoshi A, Otsuki M. 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. Kim KP, Kim MH, Lee SS, Seo DW, Lee SK. Autoimmune pancreatitis: it may be a worldwide entity. Gastroenterology. 2004;126(4):1214.

    Article  PubMed  Google Scholar 

  13. Okazaki K. Autoimmune pancreatitis is increasing in Japan. Gastroenterology. 2003;125(5):1557–8.

    Article  PubMed  Google Scholar 

  14. 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(1):129–37; discussion 37–9.

    Article  PubMed  Google Scholar 

  15. Abraham SC, Wilentz RE, Yeo CJ, Sohn TA, Cameron JL, Boitnott JK, et al. Pancreaticoduodenectomy (Whipple resections) in patients without malignancy: are they all ‘chronic pancreatitis’? Am J Surg Pathol. 2003;27(1):110–20.

    Article  PubMed  Google Scholar 

  16. 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(6):853–8; discussion 8–9.

    Google Scholar 

  17. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4(8):1010–6; quiz 934.

    Google Scholar 

  18. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A, Kamata N. Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma. Am J Gastroenterol. 2003;98(12):2694–9.

    Article  PubMed  Google Scholar 

  19. 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  CAS  PubMed  Google Scholar 

  20. Kamisawa T, Okamoto A. IgG4-related sclerosing disease. World J Gastroenterol (WJG). 2008;14(25):3948–55.

    Article  CAS  Google Scholar 

  21. 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  CAS  PubMed  Google Scholar 

  22. 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(3):244–8.

    Article  CAS  PubMed  Google Scholar 

  23. Ito T, Nakamura T, Fujimori N, Niina Y, Igarashi H, Oono T, et al. Characteristics of pancreatic diabetes in patients with autoimmune pancreatitis. J Dig Dis. 2011;12(3):210–6.

    Article  PubMed  Google Scholar 

  24. 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 

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

    Article  CAS  PubMed  Google Scholar 

  26. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30.

    Article  CAS  PubMed  Google Scholar 

  27. Kamisawa T, Nakajima H, Egawa N, Hayashi Y, Funata N. Autoimmune pancreatitis can be confirmed with gastroscopy. Dig Dis Sci. 2004;49(1):155–6.

    Article  PubMed  Google Scholar 

  28. Leise MD, Smyrk TC, Takahashi N, Sweetser SR, Vege SS, Chari ST. IgG4-associated cholecystitis: another clue in the diagnosis of autoimmune pancreatitis. Dig Dis Sci. 2011;56(5):1290–4.

    Article  CAS  PubMed  Google Scholar 

  29. Pace A, Topalidis T, Blaker M, Guthoff A, de Weerth A, Lohse AW. Autoimmune pancreatitis with normal IgG4-Levels: 4 case reports and review of the literature. Zeitschrift fur Gastroenterologie. 2007;45(12):1245–51.

    Google Scholar 

  30. Kloppel G, Detlefsen S, Chari ST, Longnecker DS, Zamboni G. Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions. J Gastroenterol. 2010;45(8):787–93.

    Article  PubMed  Google Scholar 

  31. Sugumar A, Chari ST. Distinguishing pancreatic cancer from autoimmune pancreatitis: a comparison of two strategies. Clin Gastroenterol Hepatol. 2009;7(11 Suppl):S59–62.

    Article  PubMed  Google Scholar 

  32. Kamisawa T, Takuma K, Egawa N, Tsuruta K, Sasaki T. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol. 2010;7(7):401–9.

    Article  CAS  PubMed  Google Scholar 

  33. 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  CAS  PubMed  Google Scholar 

  34. Kamisawa T, Imai M, Yui Chen P, Tu Y, Egawa N, Tsuruta K, et al. Strategy for differentiating autoimmune pancreatitis from pancreatic cancer. Pancreas. 2008;37(3):e62–7.

    Article  PubMed  Google Scholar 

  35. 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  CAS  PubMed  Google Scholar 

  36. Shin JU, Lee JK, Kim KM, Lee KH, Lee KT, Kim YK, et al. The differentiation of autoimmune pancreatitis and pancreatic cancer using imaging findings. Hepatogastroenterology. 2013;60(125):1174–81.

    PubMed  Google Scholar 

  37. 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 

  38. Chang WI, Kim BJ, Lee JK, Kang P, Lee KH, Lee KT, et al. The clinical and radiological characteristics of focal mass-forming autoimmune pancreatitis: comparison with chronic pancreatitis and pancreatic cancer. Pancreas. 2009;38(4):401–8.

    Article  PubMed  Google Scholar 

  39. Moon SH, Kim MH, Park DH, Hwang CY, Park SJ, Lee SS, et al. 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(12):1704–12.

    Article  CAS  PubMed  Google Scholar 

  40. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Morphological changes after steroid therapy in autoimmune pancreatitis. Scand J Gastroenterol. 2004;39(11):1154–8.

    Article  CAS  PubMed  Google Scholar 

  41. Gardner TB, Levy MJ, Takahashi N, Smyrk TC, Chari ST. Misdiagnosis of autoimmune pancreatitis: a caution to clinicians. Am J Gastroenterol. 2009;104(7):1620–3.

    Article  PubMed  Google Scholar 

  42. 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 

  43. Motosugi U, Ichikawa T, Yamaguchi H, Nakazawa T, Katoh R, Itakura J, et al. Small invasive ductal adenocarcinoma of the pancreas associated with lymphoplasmacytic sclerosing pancreatitis. Pathol Int. 2009;59(10):744–7.

    Article  PubMed  Google Scholar 

  44. Witkiewicz AK, Kennedy EP, Kennyon L, Yeo CJ, Hruban RH. Synchronous autoimmune pancreatitis and infiltrating pancreatic ductal adenocarcinoma: case report and review of the literature. Hum Pathol. 2008;39(10):1548–51.

    Article  PubMed  Google Scholar 

  45. 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  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kyu Taek Lee MD, PhD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Woo, Y.S., Lee, K.T. (2015). Clinical Features. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-55086-7_8

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-55085-0

  • Online ISBN: 978-3-642-55086-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics