Skip to main content
  • 915 Accesses

Abstract

Autoimmune pancreatitis (AIP) is a recently defined new disease entity with pathogenesis possibly related to autoimmunity. Originally it was characterized by impressive imaging of the pancreas, such as diffuse swelling with main pancreatic duct narrowing, serologically an elevation of serum IgG4 and an appearance of various autoantibodies, pathologically lymphoplasmacytic sclerosing pancreatitis (LPSP), and an association of various extrapancreatic organ involvement representatively such as sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, and so on. This type AIP is recently considered to be a pancreatic manifestation of the integrated concept of IgG4-related disease. On the other hand, another type of autoimmune-related pancreatitis was proposed, which is defined pathologically by idiopathic duct-centric chronic pancreatitis (IDCP) or granulocytic epithelial lesion (GEL). Recent studies have shown the clinical features of this type being quite different from the former type, suggesting distinct category. World experts agreed that they are different types of autoimmune pancreatitis and should be given nomenclatures of type 1 AIP for the former and type 2 AIP for the latter. Based on this Honolulu consensus, the International Consensus Diagnostic Criteria (ICDC) for AIP was compiled based on the consensus of world experts after a hot discussion in the symposium at the 14th IAP and 41st JPS joint conference in Fukuoka in 2010. Now we are ready to study and discuss globally on the same ground by the ICDC the pathogenesis, the clinical features, and the best treatment for AIP.

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

Access this chapter

Subscribe and save

Springer+ Basic
$34.99 /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 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

Institutional subscriptions

Similar content being viewed by others

References

  1. Okazaki K, Kawa S, Kamisawa T, Ito T, Inui K, Irie H, Irisawa A, Kubo K, Notohara K, Hasebe O, Fujinaga Y, Ohara H, Tanaka S, Nishino T, Nishimori I, Nishiyama T, Suda K, Shiratori K, Shimosegawa T, Tanaka M. Japanese clinical guidelines for autoimmune pancreatitis. Pancreas. 2009;38:849–66.

    Article  PubMed  Google Scholar 

  2. Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, Ohara H, Ito T, Kiriyama S, Inui K, Shimosegawa T, Koizumi M, Suda K, Shiratori K, Yamaguchi K, Yamaguchi T, Sugiyama M, Otsuki M. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41: 626–31.

    Article  PubMed  Google Scholar 

  3. Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, Pearson RK, Smyrk TC, Petersen BT, Topazian MD, Takahash N, Farnell MB, Vege SS. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology. 2010;139:140–8.

    Article  PubMed  Google Scholar 

  4. Kawa S, Hamano H. Clinical features of autoimmune pancreatitis. J Gastroenterol. 2007;42(Suppl XVIII):9–14.

    Article  PubMed  Google Scholar 

  5. 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 

  6. Kamisawa T, Tu Y, Nakajima H, Egawa N, Tsuruta K, Okamoto A, Horiguchi S. Sclerosing cholecystitis associated with autoimmune pancreatitis. World J Gastroenterol. 2006;12:3736–9.

    PubMed  Google Scholar 

  7. Hyodo N, Hyodo T. Ultrasonographic evaluation in patients with autoimmune-related pancreatitis. J Gastroenterol. 2003;38:1155–61.

    Article  PubMed  Google Scholar 

  8. Irie H, Honda H, Baba S, Kuroiwa T, Yoshimitsu K, Tajima T, Jimi M, Sumii T, Masuda K. Autoimmune pancreatitis: CT and MR characteristics. Am J Roentgenol. 1998;170:1323–7.

    Article  CAS  Google Scholar 

  9. Ito T, Nakano I, Koyanagi S, Miyahara T, Migita Y, Ogoshi K, Sakai H, Matsunaga S, Yasuda O, Sumii T, Nawata H. Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci. 1997;42:1458–68.

    Article  PubMed  CAS  Google Scholar 

  10. Wakabayashi T, Motoo Y, Kojima Y, Makino H, Sawabu N. Chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct. Dig Dis Sci. 1998;43:2415–25.

    Article  PubMed  CAS  Google Scholar 

  11. Horiuchi A, Kawa S, Hamano H, Hayama M, Ota H, Kiyosawa K. ERCP features in 27 patients with autoimmune pancreatitis. Gastrointest Endosc. 2002;55: 494–9.

    PubMed  Google Scholar 

  12. Kamisawa T, Tu Y, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Involvement of pancreatic and bile ducts in autoimmune pancreatitis. World J Gastroenterol. 2006;12:612–4.

    PubMed  Google Scholar 

  13. Hirano K, Shiratori Y, Komatsu Y, Yamamoto N, Sasahira N, Toda N, Isayama H, Tada M, Tsujino T, Nakata R, Kawase T, Katamoto T, Kawabe T, Omata M. Involvement of the biliary system in autoimmune pancreatitis: a follow-up study. Clin Gastroenterol Hepatol. 2003;1:453–64.

    Article  PubMed  Google Scholar 

  14. Higashi T, Saga T, Nakamoto Y, Ishimori T, Fujimoto K, Doi R, Imamura M, Konishi J. Diagnosis of pancreatic cancer using fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) – usefulness and limitations in “clinical reality”. Ann Nucl Med. 2003;17:261–79.

    Article  PubMed  Google Scholar 

  15. Ozaki Y, Oguchi K, Hamano H, Arakura N, Muraki T, Kiyosawa K, Momose M, Kadoya M, Miyata K, Aizawa T, Kawa S. Differentiation of autoimmune pancreatitis from suspected pancreatic cancer by fluorine-18 fluorodeoxyglucose positron emission tomography. J Gastroenterol. 2008;43:144–51.

    Article  PubMed  CAS  Google Scholar 

  16. Sato M, Okumura T, Shioyama Y, Imura J. Extrapancreatic F-18 FDG accumulation in autoimmune pancreatitis. Ann Nucl Med. 2008;22:215–9.

    Article  PubMed  Google Scholar 

  17. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.

    Article  PubMed  CAS  Google Scholar 

  18. 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  PubMed  CAS  Google Scholar 

  19. Okazaki K, Uchida K, Ohana M, Nakase H, Uose S, Inai M, Matsushima Y, Katamura K, Ohmori K, Chiba T. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology. 2000;118:573–81.

    Article  PubMed  CAS  Google Scholar 

  20. Asada M, Nishio A, Uchida K, Kido M, Ueno S, Uza N, Kiriya K, Inoue S, Kitamura H, Ohashi S, Tamaki H, Fukui T, Matsuura M, Kawasaki K, Nishi T, Watanabe N, Nakase H, Chiba T, Okazaki K. Identification of a novel autoantibody against pancreatic secretory trypsin inhibitor in patients with autoimmune pancreatitis. Pancreas. 2006;33:20–6.

    Article  PubMed  CAS  Google Scholar 

  21. Frulloni L, Lunardi C, Simone R, Dolcino M, Scattolini C, Falconi M, Benini L, Vantini I, Corrocher R, Puccetti A. Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med. 2009;361:2135–42.

    Article  PubMed  CAS  Google Scholar 

  22. Komatsu K, Hamano H, Ochi Y, Takayama M, Muraki T, Yoshizawa K, Sakurai A, Ota M, Kawa S. High prevalence of hypothyroidism in patients with autoimmune pancreatitis. Dig Dis Sci. 2005;50:1052–7.

    Article  PubMed  Google Scholar 

  23. 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 

  24. Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis. Hum Pathol. 1991;22:387–95.

    Article  PubMed  CAS  Google Scholar 

  25. Suda K, Takase M, Fukumura Y, Ogura K, Ueda A, Matsuda T, Suzuki F. Histopathologic characteristics of autoimmune pancreatitis based on comparison with chronic pancreatitis. Pancreas. 2005;30:355–8.

    Article  PubMed  Google Scholar 

  26. Kojima M, Sipos B, Klapper W, Frahm O, Knuth HC, Yanagisawa A, Zamboni G, Morohoshi T, Klöppel G. Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol. 2007;31:521–8.

    Article  PubMed  Google Scholar 

  27. Zhang L, Notohara K, Levy MJ, Chari ST, Smyrk TC. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2007;20:23–8.

    Article  PubMed  CAS  Google Scholar 

  28. 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 

  29. 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 biopsy specimens. Virchows Arch. 2004;445:552–63.

    Article  PubMed  Google Scholar 

  30. Klöppel 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: 787–93.

    Article  PubMed  Google Scholar 

  31. Kamisawa T, Funata N, Hayashi Y, Tsuruta K, Okamoto A, Amemiya K, Egawa N, Nakajima H. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003;52:683–7.

    Article  PubMed  CAS  Google Scholar 

  32. Saegusa H, Momose M, Kawa S, Hamano H, Ochi Y, Takayama M, Kiyosawa K, Kadoya M. Hilar and pancreatic gallium-67 accumulation is characteristic feature of autoimmune pancreatitis. Pancreas. 2003;27:20–5.

    Article  PubMed  Google Scholar 

  33. Nakazawa T, Ohara H, Yamada T, Ando H, Sano H, Kajino S, Hashimoto T, Nakamura S, Ando T, Nomura T, Joh T, Itoh M. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis. Hepatogastroenterology. 2001;48:625–30.

    PubMed  CAS  Google Scholar 

  34. Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H, Kiyosawa K. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359: 1403–4.

    Article  PubMed  Google Scholar 

  35. Takeda S, Haratake J, Kasai T, Takaeda C, Takazakura E. IgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis. Nephrol Dial Transplant. 2004;19:474–6.

    Article  PubMed  Google Scholar 

  36. Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H, Saiga T, Okamoto M, Fukuse T. Interstitial pneumonia associated with autoimmune pancreatitis. Gut. 2003;52:683–7.

    Article  Google Scholar 

  37. Taniguchi T, Hamasaki A, Okamoto M. A case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia. Endocr J. 2006;53:563–6.

    Article  PubMed  Google Scholar 

  38. Kanno A, Satoh K, Kimura K, Masamune A, Asakura T, Unno M, Matsuno S, Moriya T, Shimosegawa T. Autoimmune pancreatitis with hepatic inflammatory pseudotumor. Pancreas. 2005;31:420–3.

    Article  PubMed  Google Scholar 

  39. Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y. IgG4-realted sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis. Am J Surg Pathol. 2004;28:1193–203.

    Article  PubMed  Google Scholar 

  40. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A, Hayashi Y, Funata N. Gastrointestinal findings in patients with autoimmune pancreatitis. Endoscopy. 2005;37:1127–30.

    Article  PubMed  CAS  Google Scholar 

  41. Yoshimura Y, Takeda S, Ieki Y, Takazakura E, Koizumi H, Takagawa K. IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med. 2006;45:897–901.

    Article  PubMed  Google Scholar 

  42. Ohara H, Nakazawa T, Sano H, Ando T, Okamoto T, Takada H, Hayashi K, Kitajima Y, Nakao H, Joh T. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31:232–7.

    Article  PubMed  Google Scholar 

  43. Yamamoto M, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, Imai K. Elevated IgG4 concentrations in serum of patients with Mikulicz’s disease. Scand J Rheumatol. 2004;33:432–3.

    Article  PubMed  CAS  Google Scholar 

  44. Yamamoto M, Harada S, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, Imai K. Clinical and pathological differences between Mikulicz’s disease and Sjögren syndrome. Rheumatology. 2005;44:227–34.

    Article  PubMed  CAS  Google Scholar 

  45. Nakazawa T, Ohara H, Sano H, Aoki S, Kobayashi S, Okamoto T, Imai H, Nomura T, Joh T, Itoh M. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc. 2004;60:937–44.

    Article  PubMed  Google Scholar 

  46. Nakazawa T, Ohara H, Sano H, Ando T, Aoki S, Kobayashi S, Okamoto T, Nomura T, Joh T, Itoh M. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas. 2005;30:20–5.

    PubMed  Google Scholar 

  47. Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A. A new diagnostic endoscopic tool for autoimmune pancreatitis. Gastrointest Endosc. 2008;68:358–61.

    Article  PubMed  Google Scholar 

  48. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, Okamoto A, Egawa N, Nakajima H. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.

    Article  PubMed  CAS  Google Scholar 

  49. Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6:132–7.

    Article  PubMed  CAS  Google Scholar 

  50. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, Okamoto A, Egawa N, Nakajima H. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003;52:683–7.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  52. Sugumar A, Smyrk TC, Takahashi N, Levy MJ, Chari ST. Lymphoplasmacytic sclerosing pancreatitis (LPSP) and idiopathic duct centric pancreatitis (IDCP) are distinct clinical forms of autoimmune pancreatitis (AIP). Pancreas. 2008;37:497.

    Article  Google Scholar 

  53. 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 

  54. Kim K-P, Kim M-H, Kim JC, Lee SS, Seo DW, Lee SK. Diagnostic criteria for autoimmune chronic pancreatitis revisited. World J Gastroenterol. 2006;12: 2487–96.

    PubMed  Google Scholar 

  55. Choi EK, Kim M-H, Kim JC, Han J, Seo DW, Lee SS, Lee SK. The Japanese diagnostic criteria for autoimmune chronic pancreatitis: Is it completely satisfactory? Pancreas. 2006;33:13–9.

    Article  PubMed  Google Scholar 

  56. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–6.

    Article  PubMed  Google Scholar 

  57. Otsuki M, Chung JB, Okazaki K, Kim M-H, Kamisawa T, Kawa S, Park SW, Shimosegawa T, Lee K, Ito T, Nishimori I, Notohara K, Naruse S, Ko SBH, Ko SBH, Kihara Y, The Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labour and Welfare of Japan and the Korean Society of Pancreatobiliary Diseases. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea symposium on autoimmune pancreatitis. J Gastroenterol. 2008;43:403–8.

    Article  PubMed  CAS  Google Scholar 

  58. Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T, The Autoimmune Pancreatitis International Cooperative Study Group (APICS). Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39:549–54.

    Article  PubMed  CAS  Google Scholar 

  59. Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim M-H, Klöppel G, Lerch MM, Löhr M, Notohoara K, Okazaki K, Schneider A, Zhang L. International consensus diagnostic criteria for autoimmune pancreatitis. Guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–8.

    Article  PubMed  Google Scholar 

  60. Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, Okumura F, Nishikawa T, Kobayashi K, Ichiya T, Takatori H, Yamakita K, Kubota K, Hamano H, Okamura K, Hirano K, Ito T, Ko SBH, Omata M. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.

    Article  PubMed  CAS  Google Scholar 

  61. Ito T, Nishimori I, Inoue N, Kawabe K, Gibo J, Arita Y, Okazaki K, Takayanagi R, Otsuki M. Treatment for autoimmune pancreatitis: consensus on the treatment for patients with autoimmune pancreatitis in Japan. J Gastroenterol. 2007;42(Suppl XVIII):50–8.

    Article  PubMed  Google Scholar 

  62. Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, Nakai Y, Sasahira N, Tsujino T, Yoshida H, Kawabe T, Omata M. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut. 2007;56:1719–24.

    Article  PubMed  CAS  Google Scholar 

  63. Kamisawa T, Okamoto A, Wakabayashi T, Watanabe H, Sawabu N. Appropriate steroid therapy for autoimmune pancreatitis based on long-term outcome. Scand J Gastroenterol. 2008;43:609–13.

    Article  PubMed  CAS  Google Scholar 

  64. Church NI, Pereira S, Deheragoda MG, Sandanayake N, Amin Z, Lees WR, Gillams A, Rodriguez-Justo M, Novelli M, Seward EW, Hatfield ARW, Webster GJM. Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series. Am J Gastroenterol. 2007;102:2417–25.

    Article  PubMed  CAS  Google Scholar 

  65. Sandanayake NS, Church NI, Chapman MH, Johnson GJ, Dhar DK, Amin Z, Deheragoda MG, Novelli M, Winstanley A, Rodrizuez-Justo M, Hatfield ARW, Pereira SP, Webster GJM. Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2009;7:1089–96.

    Article  PubMed  CAS  Google Scholar 

  66. Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Witcomb DC, Slivka A. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol. 2009;104:2295–306.

    Article  PubMed  Google Scholar 

  67. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, Topazian MD, Clain JE, Pearson RK, Petersen BT, Vege SS, Lindor K, Farnell MB. Immunoglobulin G4-associate cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.

    Article  PubMed  Google Scholar 

  68. Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, Chari ST. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2008;6:364–6.

    Article  PubMed  CAS  Google Scholar 

  69. Rueda JC, Duarte-Rey C, Casas N. Successful treatment of relapsing autoimmune pancreatitis in primary Sjögren’s syndrome with rituximab: report of a case and review of the literature. Rheumatol Int. 2009;29:1481–5.

    Article  PubMed  Google Scholar 

  70. Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-realted systemic disease. Arthritis Rheum. 2010;62:1755–62.

    Article  PubMed  CAS  Google Scholar 

  71. Kamisawa T, Yoshiike M, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology. 2005;5:234–8.

    Article  PubMed  Google Scholar 

  72. Nishino T, Toki F, Oyama H, Shimizu K, Shiratori K. Long-term outcome of autoimmune pancreatitis after oral prednisolone therapy. Intern Med. 2006;45: 497–501.

    Article  PubMed  Google Scholar 

  73. Takayama M, Hamano H, Ochi Y, Saegusa H, Komatsu K, Muraki T, Arakura N, Imai Y, Hasebe O, Kawa S. Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol. 2004;99:932–7.

    Article  PubMed  Google Scholar 

  74. Iida H, Kubota K, Mawatari H, Yoneda M, Goto A, Abe Y, Inamori M, Kobayashi N, Kirikoshi H, Saito S, Nakajima A. A case of autoimmune pancreatitis developed pancreatic tail cancer. (Japanese and English abstract) Suizo. 2008;23:608–14.

    Google Scholar 

  75. Inoue H, Miyatani H, Sawada Y, Yoshida Y. A case of pancreas cancer with autoimmune pancreatitis. Pancreas. 2006;33:208–9.

    Article  PubMed  Google Scholar 

  76. Ghazale A, Chari S. Is autoimmune pancreatitis a risk factor for pancreatic cancer. Pancreas. 2007;35:376.

    Article  PubMed  Google Scholar 

  77. Bang S-J, Kim M-H, Kim DH, Lee TY, Kwon S, Oh H-C, Kim JY, Hwang CY, Lee SS, Seo DW, Kee SK, Song DE, Jang SJ. Is pancreatic core biopsy sufficient to diagnose autoimmune chronic pancreatitis? Pancreas. 2008;36:84–9.

    Article  PubMed  Google Scholar 

  78. Levy MJ, Reddy RP, Wiersema MJ, Smyrk TC, Clain JE, Harewood GC, Pearson RK, Rajan E, Topazian MD, Yusuf TE, Chari ST, Peterson BT. EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaundice. Gastrointest Endosc. 2005;61:467–72.

    Article  PubMed  Google Scholar 

  79. DeWitt J, McGreevy K, LeBlanc J, McHenry L, Cummings O, Sherman S. EUS-guided Trucut biopsy of suspected nonfocal chronic pancreatitis. Gastrointest Endosc. 2005;62:76–84.

    Article  PubMed  Google Scholar 

  80. Yoshida K, Toki F, Takeuchi T, Watanabe S-I, 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  PubMed  CAS  Google Scholar 

  81. Kamisawa T, Chari ST, Giday SA, Kim M-H, Chang JB, Lee KT, Werner J, Bergmann F, Lerch MM, Mayerle J, Pickartz T, Lohr M, Schneider A, Frulloni L, Webster GJM, Reddy ND, Liao W-C, Wang H-P, Okazaki K, Shimosegawa T, Kloeppel G, Go VLW. Clinical profile of autoimmune pancreatitis and its histological subtypes. An international multicenter survey. Pancreas. 2011;40:809–14.

    Article  PubMed  CAS  Google Scholar 

  82. Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, Levy MJ, Pearson RK, Petersen BT, Smyrk TC, Sugumar A, Takahashi N, Vege SS, Chari ST. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2012;0:1–8. doi:10.1136/gutjnl-2012-302886.

    Google Scholar 

Download references

Acknowledgment

The author is grateful to Atushi Kanno for great helps to prepare figures and to Kenji Notohara for providing the beautiful pictures of IDCP/GELs (Fig. 3.9). This manuscript is made with a support in part by the Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labour, and Welfare of Japan.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Tooru Shimosegawa M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Shimosegawa, T. (2013). Overview of Types 1 and 2. In: Levy, M., Chari, S. (eds) Autoimmune (IgG4-related) Pancreatitis and Cholangitis. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6430-4_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-4419-6430-4_3

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-6429-8

  • Online ISBN: 978-1-4419-6430-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics