Skip to main content
SpringerLink
Log in

Autoimmune Pancreatitis in the USA

  • Chapter
  • First Online:
Autoimmune Pancreatitis

  • 1089 Accesses

Abstract

Autoimmune pancreatitis (AIP) is a recently recognized disease affecting the pancreas. It closely mimics pancreatic cancer in presentation. In fact, pancreatic resections performed for presumed pancreatic cancer have historically played a key role in recognition of this disease entity. Characterized by distinct histological features in the pancreas which has formed the basis of diagnosis for a majority of patients historically, increased recognition of this disease has led to development of diagnostic criteria. In the USA, the HISORt criteria introduced in 2006 has formed the backbone of diagnosing this disease. Recently, international consensus criteria have been developed incorporating experiences and practices from across the world. With increasing experience of this disease, treatment and follow-up strategies have now emerged. The disease is characteristically responsive to steroid treatment. However, relapses are common. Interestingly, two groups of AIP patients were observed with distinct patterns of relapse and corresponding distinctive histological and clinical features. This led to subclassification of AIP into two subtypes: type 1 in which relapses are common and histologically corresponding to lymphoplasmacytic sclerosing pancreatitis (LPSP) with IgG4 positivity and type 2 in which relapses are rare and histologically characterized by granulocyte epithelial lesions (GELs). Long-term follow-up data have now established that despite relapses, AIP is a benign disease which does not affect overall survival.

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

Access this chapter

Log in via an institution
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

References

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

    Article  CAS  PubMed  Google Scholar 

  2. Sarles H, Sarles JC, Camatte R, et al. Observations on 205 confirmed cases of acute pancreatitis, recurring pancreatitis, and chronic pancreatitis. Gut. 1965;6(6):545–59.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Sarles H, Adler G, Dani R, et al. The pancreatitis classification of Marseilles-Rome 1988. Scand J Gastroenterol. 1989;24(6):641–2.

    Article  CAS  PubMed  Google Scholar 

  4. Yoshida K, Toki F, Takeuchi T, et al. 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. Ectors N, Maillet B, Aerts R, et al. Non-alcoholic duct destructive chronic pancreatitis. Gut. 1997;41(2):263–8.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  6. Society MotCCfAPotJP. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society. J Jpn Pancreas Soc. 2002;17:585–7.

    Google Scholar 

  7. Uchida K, Okazaki K, Konishi Y, et al. Clinical analysis of autoimmune-related pancreatitis. Am J Gastroenterol. 2000;95(10):2788–94.

    Article  CAS  PubMed  Google Scholar 

  8. Okazaki K, Uchida K, Ohana M, et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology. 2000;118(3):573–81.

    Article  CAS  PubMed  Google Scholar 

  9. Tanaka S, Kobayashi T, Nakanishi K, et al. Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis. Lancet. 2000;356(9233):910–1.

    Article  CAS  PubMed  Google Scholar 

  10. Ito T, Nakano I, Koyanagi S, et al. Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci. 1997;42(7):1458–68.

    Article  CAS  PubMed  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(10):732–8.

    Article  CAS  PubMed  Google Scholar 

  12. Bartholomew LG, Cain JC, Woolner LB, et al. Sclerosing cholangitis: its possible association with Riedel’s struma and fibrous retroperitonitis. Report of two cases. N Engl J Med. 1963;269:8–12.

    Article  CAS  PubMed  Google Scholar 

  13. Hache L, Woolner LB, Bernatz PE. Idiopathic fibrous mediastinitis. Dis Chest. 1962;41:9–25.

    Article  CAS  PubMed  Google Scholar 

  14. Wenger J, Gingrich GW, Mendeloff J. Sclerosing cholangitis – a manifestation of systemic disease. Increased serum gamma-globulin, follicular lymph node hyperplasia, and orbital pseudotumor. Arch Intern Med. 1965;116(4):509–14.

    Article  CAS  PubMed  Google Scholar 

  15. Comings DE, Skubi KB, Van Eyes J, et al. Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med. 1967;66(5):884–92.

    Article  CAS  PubMed  Google Scholar 

  16. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 6-1982. A 55-year-old man with eight months of obstructive jaundice. N Engl J Med. 1982;306(6):349–58.

    Google Scholar 

  17. Meijer S, Hoitsma HF, Scholtmeijer R. Idiopathic retroperitoneal fibrosis in multifocal fibrosclerosis. Eur Urol. 1976;2(5):258–60.

    CAS  PubMed  Google Scholar 

  18. Meyer S, Hausman R. Occlusive phlebitis in multifocal fibrosclerosis. Am J Clin Pathol. 1976;65(3):274–83.

    CAS  PubMed  Google Scholar 

  19. Waldram R, Kopelman H, Tsantoulas D, et al. Chronic pancreatitis, sclerosing cholangitis, and sicca complex in two siblings. Lancet. 1975;1(7906):550–2.

    Article  CAS  PubMed  Google Scholar 

  20. Sood S, Fossard DP, Shorrock K. Chronic sclerosing pancreatitis in Sjogren’s syndrome: a case report. Pancreas. 1995;10(4):419–21.

    Article  CAS  PubMed  Google Scholar 

  21. Clark A, Zeman RK, Choyke PL, et al. Pancreatic pseudotumors associated with multifocal idiopathic fibrosclerosis. Gastrointest Radiol. 1988;13(1):30–2.

    Article  CAS  PubMed  Google Scholar 

  22. Scully KA, Li SC, Hebert JC, et al. The characteristic appearance of non-alcoholic duct destructive chronic pancreatitis: a report of 2 cases. Arch Pathol Lab Med. 2000;124(10):1535–8.

    CAS  PubMed  Google Scholar 

  23. Etemad B, Whitcomb DC. Chronic pancreatitis: diagnosis, classification, and new genetic developments. Gastroenterology. 2001;120(3):682–707.

    Article  CAS  PubMed  Google Scholar 

  24. Notohara K, Burgart LJ, Yadav D, et al. 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 

  25. Weber SM, Cubukcu-Dimopulo O, Palesty JA, 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 

  26. Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, et al. Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg. 2003;237(6):853–8; discussion 8–9.

    PubMed Central  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  29. Raina A, Yadav D, Krasinskas AM, 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 

  30. Clark CJ, Morales-Oyarvide V, Zaydfudim V, et al. Short-term and long-term outcomes for patients with autoimmune pancreatitis after pancreatectomy: a multi-institutional study. J Gastrointest Surg. 2013;17(5):899–906.

    Article  PubMed  Google Scholar 

  31. Kim MH, Kwon S. Diagnostic criteria for autoimmune chronic pancreatitis. J Gastroenterol. 2007;42 Suppl 18:42–9.

    Article  PubMed  Google Scholar 

  32. Schneider A, Lohr JM, Singer MV. The M-ANNHEIM classification of chronic pancreatitis: introduction of a unifying classification system based on a review of previous classifications of the disease. J Gastroenterol. 2007;42(2):101–19.

    Article  PubMed  Google Scholar 

  33. 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(3):249–65.

    Article  PubMed  Google Scholar 

  34. 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(6):403–8.

    Article  CAS  PubMed  Google Scholar 

  35. Gardner TB, Levy MJ, Takahashi N, et al. Misdiagnosis of autoimmune pancreatitis: a caution to clinicians. Am J Gastroenterol. 2009;104(7):1620–3.

    Article  PubMed  Google Scholar 

  36. Chari ST, Takahashi N, Levy MJ, 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 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  39. Levy MJ, Smyrk TC, Takahashi N, et al. Idiopathic duct-centric pancreatitis: disease description and endoscopic ultrasonography-guided trucut biopsy diagnosis. Pancreatology. 2011;11(1):76–80.

    Article  PubMed  Google Scholar 

  40. 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(3):352–8.

    Article  PubMed  Google Scholar 

  41. 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(9):2288–94.

    Article  PubMed  Google Scholar 

  42. Church NI, Pereira SP, Deheragoda MG, et al. Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series. Am J Gastroenterol. 2007;102(11):2417–25.

    Article  CAS  PubMed  Google Scholar 

  43. Zamboni G, Luttges J, Capelli P, et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445(6):552–63.

    Article  PubMed  Google Scholar 

  44. Ryu JK, Chung JB, Park SW, et al. Review of 67 patients with autoimmune pancreatitis in Korea: a multicenter nationwide study. Pancreas. 2008;37(4):377–85.

    Article  PubMed  Google Scholar 

  45. Maire F, Le Baleur Y, Rebours V, 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 

  46. Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134(3):706–15.

    Article  PubMed  Google Scholar 

  47. Sah RP, Chari ST, Pannala R, 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.

    Article  PubMed  Google Scholar 

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

    Article  PubMed Central  PubMed  Google Scholar 

  49. Kamisawa T, Funata N, Hayashi Y, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003;52(5):683–7.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  51. Kamisawa T, Funata N, Hayashi Y. Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of IgG4-related systemic disease. Am J Surg Pathol. 2004;28(8):1114.

    Article  PubMed  Google Scholar 

  52. Kamisawa T, Nakajima H, Egawa N, et al. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6(1–2):132–7.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  54. Stone JH, Khosroshahi V, Deshpande V. IgG4-related disease: recommendations for the nomenclature of this condition and its individual organ system manifestations. Arthritis Rheum. 2012;64(10):3061–7.

    Article  CAS  PubMed  Google Scholar 

  55. Ghazale A, Chari ST, Smyrk TC, 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 

  56. Moon SH, Kim MH, Park DH, 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 

  57. 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 

  58. 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 

  59. Ghazale A, Chari ST. Optimising corticosteroid treatment for autoimmune pancreatitis. Gut. 2007;56(12):1650–2.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  60. Hart PA, Topazian MD, Witzig TE, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2013;62(11):1607–15.

    Article  CAS  PubMed  Google Scholar 

  61. Hart PA, Law RJ, Dierkhising RA, et al. 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 

  62. Kamisawa T, Chari ST, Giday SA, 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 

  63. Levy MJ, Reddy RP, Wiersema MJ, et al. EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaundice. Gastrointest Endosc. 2005;61(3):467–72.

    Article  PubMed  Google Scholar 

  64. Levy MJ. Endoscopic ultrasound-guided trucut biopsy of the pancreas: prospects and problems. Pancreatology. 2007;7(2–3):163–6.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  66. Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, 55905, USA

    Raghuwansh P. Sah MD, Nazir Ahmed MD, Philip A. Hart MD & Suresh T. Chari MD

Authors
  1. Raghuwansh P. Sah MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nazir Ahmed MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Philip A. Hart MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Suresh T. Chari MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Suresh T. Chari MD .

Editor information

Editors and Affiliations

  1. Department of Internal Medicine, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Terumi Kamisawa

  2. Department of Internal Medicine, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea, Republic of (South Korea)

    Jae Bock Chung

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Sah, R.P., Ahmed, N., Hart, P.A., Chari, S.T. (2015). Autoimmune Pancreatitis in the USA. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_28

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-55086-7_28

  • 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

Search

Navigation