Skip to main content

Autoimmune (IgG4-Related) Pancreatitis

  • Chapter
  • First Online:
  • 674 Accesses

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Autoimmune pancreatitis (AIP) is a peculiar form of chronic inflammatory disease of the pancreas with unique pathologic features, different from all other forms of pancreatitis. Although a possible autoimmune involvement of the pancreatic gland had been proposed since the early 1960s, the term AIP was introduced in 1995 by Yoshida et al. based on a case report and the review of the available English and Japanese literature. However only in recent years was AIP finally accepted as a distinct nosographic entity. The more relevant clinical data and the main reason for introducing the term “autoimmune pancreatitis” were the dramatic response to steroid therapy, which represents one of the cardinal diagnostic criteria, like other gastrointestinal tract diseases (e.g., autoimmune hepatitis).

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   169.99
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

Learn about institutional subscriptions

References

  1. Sarles H et al (1961) Chronic inflammatory sclerosis of the pancreas–an autonomous pancreatic disease? Am J Dig Dis 6:688–698

    Article  CAS  PubMed  Google Scholar 

  2. Yoshida K et al (1995) Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 40(7):1561–1568

    Article  CAS  PubMed  Google Scholar 

  3. Hart PA, Zen Y, Chari ST (2015) Recent advances in autoimmune pancreatitis. Gastroenterology 149(1):39–51

    Article  CAS  PubMed  Google Scholar 

  4. Frulloni L, Lunardi C (2011) Serum IgG4 in autoimmune pancreatitis: a marker of disease severity and recurrence? Dig Liver Dis 43(9):674–675

    Article  PubMed  Google Scholar 

  5. Maire F et al (2011) Outcome of patients with type 1 or 2 autoimmune pancreatitis. Am J Gastroenterol 106(1):151–156

    Article  CAS  PubMed  Google Scholar 

  6. Frulloni L et al (2009) Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol 104(9):2288–2294

    Article  PubMed  Google Scholar 

  7. Okazaki K et al (2011) Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol 46(3):277–288

    Article  CAS  PubMed  Google Scholar 

  8. Zamboni G et al (2004) Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch 445(6):552–563

    Article  PubMed  Google Scholar 

  9. Frulloni L et al (2011) A practical approach to the diagnosis of autoimmune pancreatitis. World J Gastroenterol 17(16):2076–2079

    Article  PubMed  PubMed Central  Google Scholar 

  10. Vitali F et al (2014) Frequency and characterization of benign lesions in patients undergoing surgery for the suspicion of solid pancreatic neoplasm. Pancreas 43(8):1329–1333

    Article  PubMed  Google Scholar 

  11. Kim KP et al (2006) Diagnostic criteria for autoimmune chronic pancreatitis revisited. World J Gastroenterol 12(16):2487–2496

    Article  PubMed  PubMed Central  Google Scholar 

  12. Chari ST et al (2010) Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas 39(5):549–554

    Article  CAS  PubMed  Google Scholar 

  13. Frulloni L et al (2009) Chronic pancreatitis: report from a multicenter Italian survey (PanCroInfAISP) on 893 patients. Dig Liver Dis 41(4):311–317

    Article  CAS  PubMed  Google Scholar 

  14. Hart PA et al (2013) Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut 62(12):1771–1776

    Article  PubMed  Google Scholar 

  15. Nishimori I, Tamakoshi A, Otsuki M (2007) Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol 42(Suppl 18):6–8

    Article  PubMed  Google Scholar 

  16. Kanno A et al (2012) Nationwide epidemiological survey of autoimmune pancreatitis in Japan. Pancreas 41(6):835–839

    Article  PubMed  Google Scholar 

  17. Ikeura T et al (2013) Application of international consensus diagnostic criteria to an Italian series of autoimmune pancreatitis. United European Gastroenterol J 1(4):276–284

    Article  PubMed  PubMed Central  Google Scholar 

  18. Stone JH, Zen Y, Deshpande V (2012) IgG4-related disease. N Engl J Med 366(6):539–551

    Article  CAS  PubMed  Google Scholar 

  19. Shimosegawa T et al (2011) International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 40(3):352–358

    Article  PubMed  Google Scholar 

  20. Chari ST (2007) Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J Gastroenterol 42(Suppl 18):39–41

    Article  PubMed  Google Scholar 

  21. Choi EK et al (2006) The Japanese diagnostic criteria for autoimmune chronic pancreatitis: is it completely satisfactory? Pancreas 33(1):13–19

    Article  PubMed  Google Scholar 

  22. Otsuki M et al (2008) Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. J Gastroenterol 43(6):403–408

    Article  CAS  PubMed  Google Scholar 

  23. Sah RP, Chari ST (2011) Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol 23(1):108–113

    Article  CAS  PubMed  Google Scholar 

  24. Ghazale A et al (2007) Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 102(8):1646–1653

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  26. Frulloni L et al (2009) Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med 361(22):2135–2142

    Article  CAS  PubMed  Google Scholar 

  27. Manfredi R et al (2008) Autoimmune pancreatitis: CT patterns and their changes after steroid treatment. Radiology 247(2):435–443

    Article  PubMed  Google Scholar 

  28. Procacci C et al (2001) Autoimmune pancreatitis: possibilities of CT characterization. Pancreatology 1(3):246–253

    Article  CAS  PubMed  Google Scholar 

  29. Rehnitz C et al (2011) Morphologic patterns of autoimmune pancreatitis in CT and MRI. Pancreatology 11(2):240–251

    Article  PubMed  Google Scholar 

  30. Suzuki K et al (2010) CT findings in autoimmune pancreatitis: assessment using multiphase contrast-enhanced multisection CT. Clin Radiol 65(9):735–743

    Article  CAS  PubMed  Google Scholar 

  31. Manfredi R et al (2011) Autoimmune pancreatitis: pancreatic and extrapancreatic MR imaging-MR cholangiopancreatography findings at diagnosis, after steroid therapy, and at recurrence. Radiology 260(2):428–436

    Article  PubMed  Google Scholar 

  32. Carbognin G et al (2009) Autoimmune pancreatitis: imaging findings on contrast-enhanced MR, MRCP and dynamic secretin-enhanced MRCP. Radiol Med 114(8):1214–1231

    Article  CAS  PubMed  Google Scholar 

  33. Eerens I et al (2001) Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings. Eur Radiol 11(8):1401–1404

    Article  CAS  PubMed  Google Scholar 

  34. Irie H et al (1998) Autoimmune pancreatitis: CT and MR characteristics. AJR Am J Roentgenol 170(5):1323–1327

    Article  CAS  PubMed  Google Scholar 

  35. Negrelli R et al (2015) Pancreatic duct abnormalities in focal autoimmune pancreatitis: MR/MRCP imaging findings. Eur Radiol 25(2):359–367

    Article  PubMed  Google Scholar 

  36. D’Onofrio M et al (2007) Contrast-enhanced ultrasonography of the pancreas. JOP 8(1 Suppl):71–76

    PubMed  Google Scholar 

  37. D’Onofrio M et al (2006) Mass-forming pancreatitis: value of contrast-enhanced ultrasonography. World J Gastroenterol 12(26):4181–4184

    Article  PubMed  PubMed Central  Google Scholar 

  38. Farrell JJ et al (2004) EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc 60(6):927–936

    Article  PubMed  Google Scholar 

  39. Itokawa F et al (2011) EUS elastography combined with the strain ratio of tissue elasticity for diagnosis of solid pancreatic masses. J Gastroenterol 46(6):843–853

    Article  PubMed  Google Scholar 

  40. D’Onofrio M et al (2004) Contrast-enhanced ultrasonography in the characterization of pancreatic mucinous cystadenoma. J Ultrasound Med 23(8):1125–1129

    PubMed  Google Scholar 

  41. Kamisawa T et al (2011) Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas 40(6):809–814

    Article  CAS  PubMed  Google Scholar 

  42. Stone JH et al (2012) Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 64(10):3061–3067

    Article  CAS  PubMed  Google Scholar 

  43. Hart PA et al (2013) Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut 62(11):1607–1615

    Article  CAS  PubMed  Google Scholar 

  44. Khosroshahi A et al (2015) International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol 67(7):1688–1699

    Article  CAS  PubMed  Google Scholar 

  45. Kawa S et al (2009) Long-term follow-up of autoimmune pancreatitis: characteristics of chronic disease and recurrence. Clin Gastroenterol Hepatol 7(11 Suppl):S18–S22

    Article  CAS  PubMed  Google Scholar 

  46. Hart PA et al (2014) Risk of cancer in autoimmune pancreatitis: a case-control study and review of the literature. Pancreas 43(3):417–421

    Article  PubMed  Google Scholar 

  47. Shiokawa M et al (2013) Risk of cancer in patients with autoimmune pancreatitis. Am J Gastroenterol 108(4):610–617

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Luca Frulloni MD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Frulloni, L., Amodio, A. (2017). Autoimmune (IgG4-Related) Pancreatitis. In: Vaglio, A. (eds) Systemic Fibroinflammatory Disorders. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-41349-5_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-41349-5_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-41347-1

  • Online ISBN: 978-3-319-41349-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics