Skip to main content

Diagnostic Criteria of Autoimmune Pancreatitis

  • Chapter
  • First Online:
Autoimmune Pancreatitis

Abstract

Autoimmune pancreatitis (AIP) is a relatively rare disease that has recently emerged as a unique type of pancreatitis with a presumed autoimmune etiology. Sarles et al. [1] first reported a form of idiopathic chronic pancreatitis which might be caused by an autoimmune mechanism in 1961. It is uncertain whether the pancreatitis they reported coincides with AIP as we currently understand it. In 1991, Kawaguchi et al. [2] reported two cases of unusual inflammatory disease, involving the pancreas and biliary tract that were resected on suspicion of pancreatic cancer, and described the histology as lymphoplasmacytic sclerosing pancreatitis (LPSP). In 1992, Toki et al. [3] reported four cases with an unusual type of chronic pancreatitis showing diffuse, irregular narrowing of the entire main pancreatic duct (MPD) on endoscopic retrograde pancreatography (ERP) scans. This type of pancreatitis was described in the marginal notes of the newly established the JPS criteria for chronic pancreatitis in 1995 as “duct-narrowing chronic pancreatitis,” a chronic inflammation of the pancreas which may not fulfill the criteria of chronic pancreatitis [4]. In 1995, Yoshida et al. [5] proposed AIP as a diagnostic entity. They summarized the clinical features as follows: increased serum γ-globulin or immunoglobulin (Ig)G levels and the presence of autoantibodies; diffuse irregular narrowing of MPD and enlargement of the pancreas; occasional association with stenosis of the lower bile duct and other autoimmune diseases; mild symptoms, usually without acute attacks of pancreatitis; effectiveness of steroid therapy; and histological findings of LPSP [5, 6]. Thereafter, AIP has been extensively reported worldwide including from Japan, Korea, Europe, and the USA. In this chapter, we will describe the diagnostic criteria of AIP, mainly focusing on the International Consensus Diagnostic Criteria (ICDC) [7] and those proposed by the JPS [8–10].

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

Access this chapter

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
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

Similar content being viewed by others

References

  1. Sarles H, Sarles JC, Muratoren R, et al. Chronic inflammatory sclerosing of the pancreas–an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–98.

    Article  CAS  PubMed  Google Scholar 

  2. Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–95.

    Article  CAS  PubMed  Google Scholar 

  3. Toki F, Kozu T, Oi I, Nakasako T, Suzuki M, Hanyu F. An unusual type of chronic pancreatitis showing diffuse irregular narrowing of the entire main pancreatic duct on ERCP-a report of four cases. Endoscopy. 1992;24:640.

    Google Scholar 

  4. The Criteria Committee for Chronic Pancreatitis of the Japan Pancreas Society. Final report of clinical diagnostic criteria of chronic pancreatitis (in Japanese). Suizo. 1995;10:xxiii–vi.

    Google Scholar 

  5. 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;40:1561–8.

    Article  CAS  PubMed  Google Scholar 

  6. Kamisawa T, Notohara K, Shimosegawa T. Two clinicopathologic subtypes of autoimmune pancreatitis: LPSP and IDCP. Gastroenterology. 2010;139:22–5.

    Article  PubMed  Google Scholar 

  7. Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–8.

    Article  PubMed  Google Scholar 

  8. Members of the Criteria Committee for Autoimmune Pancreatitis of the Japan Pancreas Society. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society (2002). (in Japanese with English abstract) Suizo. 2002;17:585–7.

    Google Scholar 

  9. Members of the Autoimmune Pancreatitis Diagnostic Criteria Committee, the Research Committee of Intractable Diseases of the Pancreas supported by the Japanese Ministry of Health, Labor and Welfare, and Members of the Autoimmune Pancreatitis Diagnostic Criteria Committee, the Japan Pancreas Society. Clinical diagnostic criteria of autoimmune pancreatitis 2006. (in Japanese with English abstract) Suizo. 2006;21:395–7.

    Google Scholar 

  10. The Japan Pancreas Society, the Ministry of Health and Welfare Investigation Research Team for Intractable Pancreatic Disease. Clinical diagnostic for autoimmune pancreatitis 2011 (in Japanese with English abstract). Suizo. 2012;27:17–25.

    Google Scholar 

  11. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.

    Article  CAS  PubMed  Google Scholar 

  12. Okazaki K, Kawa S, Kamisawa T, et al. Japanese consensus guidelines for management of autoimmune pancreatitis: I. Concept and diagnosis of autoimmune pancreatitis. J Gastroenterol. 2010;45:249–65.

    Article  PubMed  Google Scholar 

  13. Kim KP, Kim MH, Kim JC, et al. Diagnostic criteria for autoimmune chronic pancreatitis revised. World J Gastroenterol. 2006;12:2487–96.

    PubMed Central  PubMed  Google Scholar 

  14. Chari ST, Smyrk TC, Levy MJ, et al. Autoimmune pancreatitis: diagnosis using histology, imaging, serology, other organ involvement and response to steroids. Clin Gastroenterol Hepatol. 2006;4:1010–6.

    Article  PubMed  Google Scholar 

  15. Chari ST, Takahashi N, Levy MJ, et al. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clin Gastroenterol Hepatol. 2009;10:1097–103.

    Article  Google Scholar 

  16. Schneider A, Löhr JM, Singer MV. The M-ANNHEIM classification of chronic pancreatitis: introduction of a unifying classification system based on a review of previous classification of the disease. J Gastroenterol. 2007;42:101–19.

    Article  PubMed  Google Scholar 

  17. Pearson RK, Longnecker DS, Chari ST, et al. Controversies in clinical pancreatology. Autoimmune pancreatitis: does it exist? Pancreas. 2003;27:1–13.

    Article  PubMed  Google Scholar 

  18. Frulloni L, Scattolini C, Falconi M, et al. Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol. 2009;104:2288–94.

    Article  PubMed  Google Scholar 

  19. 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;27:1119–27.

    Article  PubMed  Google Scholar 

  20. Zamboni G, Lüttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Klöppel 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.

    Article  PubMed  Google Scholar 

  21. Sugumar A, Klöppel G, Chari ST. Autoimmune pancreatitis: pathologic subtypes and their implications for its diagnosis. Am J Gastroenterol. 2009;104:2308–10.

    Article  PubMed  Google Scholar 

  22. Otsuki M, Chung JB, Okazaki K, et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea symposium on autoimmune pancreatitis. J Gastroenterol. 2008;43:403–8.

    Article  CAS  PubMed  Google Scholar 

  23. Chari ST, Klöppel G, Zhang L, et al. Histologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39:549–54.

    Article  CAS  PubMed  Google Scholar 

  24. Chari ST, Klöppel G, Zhang L, et al. Histologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreatology. 2010;10:664–72.

    Article  PubMed  Google Scholar 

  25. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.

    Article  CAS  PubMed  Google Scholar 

  26. Okazaki K, Shimosegawa T, The working Group Members of the Japan Pancreas Society and the Research Committee for Intractable Pancreatic Disease by the Ministry of Labor, Health and Welfare of Japan. The Amendment of the clinical diagnostic criteria in Japan (JPS2011) in response to the proposal of the international consensus of diagnostic criteria (ICDC) for autoimmune pancreatitis. Pancreas. 2012;41:1341–2.

    Article  Google Scholar 

  27. Sumimoto K, Uchida K, Mitsuyama T, et al. A proposal of a diagnostic algorithm with validation of International Consensus Diagnostic Criteria for autoimmune pancreatitis in a Japanese cohort. Pancreatology. 2013;13:230–7.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  29. Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41:613–25.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  31. Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Clain JE, Pearson RK, Pelaez-Luna M, Petersen BT, Vege SS, Farnell MB. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol. 2007;102:1646–53.

    Article  CAS  PubMed  Google Scholar 

  32. 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;98:2694–9.

    Article  PubMed  Google Scholar 

  33. Sahani DV, Kalva SP, Farrell J, Maher MM, Saini S, Mueller PR, Lauwers GY, Fernandez CD, Warshaw AL, Simeone JF. Autoimmune pancreatitis: imaging features. Radiology. 2004;233:345–52.

    Article  PubMed  Google Scholar 

  34. Bodily KD, Takahashi N, Fletcher JG, Fidler JL, Hough DM, Kawashima A, Chari ST. Autoimmune pancreatitis: pancreatic and extrapancreatic imaging findings. Am J Roentgenol. 2009;192:431–7.

    Article  Google Scholar 

  35. Kamisawa T, Takuma K, Anjiki H, Egawa N, Hata T, Kurata M, Honda G, Tsuruta K, Suzuki M, Kamata N, Sasaki T. Differentiation of autoimmune pancreatitis from pancreatic cancer by diffusion-weighted MRI. Am J Gastroenterol. 2010;105:1870–5.

    Article  PubMed  Google Scholar 

  36. Kanno A, Ishida K, Hamada S, Fujishima F, Unno J, Kume K, Kikuta K, Hirota M, Masamune A, Satoh K, Notohara K, Shimosegawa T. Diagnosis of autoimmune pancreatitis by EUS-FNA by using a 22-gauge needle based on the International Consensus Diagnostic Criteria. Gastrointest Endosc. 2012;76:594–602.

    Article  PubMed  Google Scholar 

  37. Ishikawa T, Itoh A, Kawashima H, Ohno E, Matsubara H, Itoh Y, Nakamura Y, Hiramatsu T, Nakamura M, Miyahara R, Ohmiya N, Goto H, Hirooka Y. Endoscopic ultrasound-guided fine needle aspiration in the differentiation of type 1 and type 2 autoimmune pancreatitis. World J Gastroenterol. 2012;18:3883–8.

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Acknowledgment

This work was supported in part by the Research Committee of Intractable Pancreatic Disease (principal investigator: Tooru Shimosegawa) provided by the Ministry of Health, Labor, and Welfare of Japan.

Disclosure

All authors disclosed no financial relationships relevant to this publication.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Atsushi Kanno 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

Kanno, A., Masamune, A., Shimosegawa, T. (2015). Diagnostic Criteria of Autoimmune Pancreatitis. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_7

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-55086-7_7

  • 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