Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2014, 158(1):017-022 | DOI: 10.5507/bp.2013.094

Autoimmune pancreatitis

Petr Ditea, Hana Nechutovab,c, Magdalena Uvirovad, Jana Dvorackovaa,e, Bohuslav Kianickab, Arnost Martineka
a Academic Center of Gastroenterology, Department of Internal Medicine, University Hospital Ostrava and Faculty of Medicine, University of Ostrava, Czech Republic
b 2nd Department of Internal Medicine, St. Anne's University Hospital Brno and Medical Faculty, Masaryk University, Brno
c International Clinical Research Center, St. Anne's University Hospital Brno
d Agel Research and Training Institute, CGB Laboratory, Ostrava-Vitkovice Branch
e Department of Pathology, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

Introduction: Autoimmune pancreatitis (AIP) is the specific type of chronic pancreatitis due to autoimmune background and mechanism.

Characteristics. The main clinical symptoms of AIP are obstructive jaundice and abdominal discomfort. The typical histological findings are lymphocytes and IgG4 plasma cells infiltration, fibrosis and venulitis within pancreatic gland. Plasma level of IgG4 is usually extremely high.

Objectives: Diagnosis: High level IgG4 positive plasma cells in serum, lymphoplasmatic infiltration found on histological staining of pancreatic tissue, "sausage-like" pancreas in ultrasound and CT scans, and response to steroid therapy are crucial for making of diagnosis.

Classification of AIP: AIP can be classified into two subtypes. Type 1 was recognized as the pancreatic manifestation of multiorgan disorder, called IgG4 related disease. Type 2 is a pancreas-specific disorder not associated with IgG4, with similar histological signs as type 1, but also with the positivity of GEL (granulocythic epithelial lesion).

Results: Therapy: Due to its high effectivity in AIP treatment, steroid therapy is the first-line option. The alternative therapy is using immunosuppressants (azathioprine). Recently, there are also first experience in biological therapy already published.

Conslusion. Before the start of AIP therapy - the differential diagnosis between pancreatic cancer and AIP is essential.

Keywords: chronic pancreatitis, autoimmune pancreatitis, immunoglobulines, IgG4, steroids, extrapancreatic lesions, pancreatic biopsy

Received: June 29, 2013; Accepted: December 13, 2013; Prepublished online: February 24, 2014; Published: April 1, 2014  Show citation

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Dite, P., Nechutova, H., Uvirova, M., Dvorackova, J., Kianicka, B., & Martinek, A. (2014). Autoimmune pancreatitis. Biomedical papers158(1), 017-022. doi: 10.5507/bp.2013.094
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References

  1. Andersen BN, Pedersen NT, Scheel J, Worning H. Incidence of alcoholic chronic pancreatitis in Copenhagen. Scand J Gastroenterol 1982,17:247-52. Go to original source... Go to PubMed...
  2. De las Heras G, Pns F. Epidemiologia y aspectos etiopatogénicos de la pancreatitis alcohólica crónica. Res Esp Enferm Dig 1993,84:253-8.
  3. Lankisch PG, Assmus C, Maisonneuve P, Lowenfels AB. Epidemiology of pancreatic diseases in Luneburg Country. A study in a defined German population. Pancreatology 2002,2:469-77. Go to original source... Go to PubMed...
  4. Dite P, Starý K, Novotný I, Precechtelová M, Dolina J, Lata J, Zboril V. Incidence of chronic pancreatitis in Czech Republic. Eur J Gastroenterol 2001,13:749-50. Go to original source...
  5. Navarro S, Soriano A. Epidemiology of chronic pancreatitis : an infrequent disease or an infrequently diagnosed disease? In Dominguez - Munoz JE (ed.). Clinical Pancreatology for Practising Gastroenterologists and Surgeons. Blackwell, 2004,pp. 187-91. Go to original source...
  6. Spanier BMW, Dijkgraaf MGW, Bruno MJ. Epidemiology, aetiology and outcome of acute and chronic pancreatitis. An update. Best Practice Res Clin Gastroenterol 2008,22,(1):45-53. Go to original source...
  7. Etemad B, Whitcomb DC. Chronic pancreatitis: diagnosis, classification, and new genetic developments. Gastroenterology 2001,120:682-97. Go to original source... Go to PubMed...
  8. Sarles H, Sarles JC, Muratore R, Guien C. Chronic anflammatory sclerosis of the pancras - an atonomous pancreatic disease? Am J Dig Dis 1961,6:688-98. Go to original source... Go to PubMed...
  9. 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, 40:1561-68. Go to original source... Go to PubMed...
  10. Okazaki K, Uchida K, Chiba T. Recent concept of autoimmune related pancreatitis. J Gastroenterol 2001,36:293-302. Go to original source... Go to PubMed...
  11. Okazaki O, Uchida K, Fukui T. Recent advances in autoimmune pancreatitis: concept, diagnosis, and pathogenesis. J Gastroenterology 2008,43:409-18. Go to original source... Go to PubMed...
  12. Nishimori I, Tamakoshi A, Otsuki M. Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol 2007:42(Suppl18):6-8 . Go to original source... Go to PubMed...
  13. Otsuki M. Autoimmune pancreatitis: a message from Japan. J Gastroenerol 2007:42(Suppl18):1-5. Go to original source...
  14. Notohara K, Brgart LJ, Yadav D. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol 1991,22:1119-27. Go to original source...
  15. Zamboni G, Lüttges J, Capelli P. 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. Go to original source... Go to PubMed...
  16. 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. Go to original source... Go to PubMed...
  17. Yamamoto M, Takahashi H, Sugai S, Imai K. Clinical and pathological characteristics of Mikulicz´s disease (IgG4-related plasmacytic exocrinopathy). Autoimmun Rev 2005,4:195-200. Go to original source... Go to PubMed...
  18. Komatsu K, Hamano H, Ochi Y, Takayama M, Muraki T, Yoshizawa K. High prevalence of hypothyroidism in patients with autoimmune pancreatitis. Dig Dis Sci 2005,50:1052-105. Go to original source... Go to PubMed...
  19. Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H. Interstitial pneumonia associated with autoimmune pancratitis. Gut 2004,53:770.
  20. Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007,102:1646-53. Go to original source... Go to PubMed...
  21. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Extrapancreatic lesions in autoimmune pancreatitis. J Clin Gastroenterol 2005,39:904-7. Go to original source... Go to PubMed...
  22. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006,4:1010-6. Go to original source... Go to PubMed...
  23. Kim MH, Lee TY. Diagnostic criteria for autoimmune pancreatitis: a proposal of revised Kim criteria. J Gastroenterol Hepatol 2007,22:A104.
  24. Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006,41:626-31. Go to original source... Go to PubMed...
  25. Naitoh I, Nakazawa T, Ohara H, Ando T, Hayashi K, Okumura F, Miyabe K, Yoshida M, Sano H, Takada H, Kanematsu T, Joh T. Comparative evaluation of the Japanese Diagnostic Criteria for autoimmune pancreatitis. Pancreas 2010,39:1173-9. Go to original source... Go to PubMed...
  26. Chari ST, Takahahi N, Levy MJ, Smyrk TC, Clain JE, Pearson RK, Petersen BT, Topazian MA, Vege SS. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clin Gastoenterol Hepatol 2009,7:1097-103. Go to original source... Go to PubMed...
  27. Inoue H, Miyatani H, Sawada Y, Yoshida Y. A case of pancreas cancer with autoimmune pancreatitis. Pancreas 2006,33:208-9. Go to original source... Go to PubMed...
  28. Fukuj T, Mitsuyama T, Takaoka M, Uchida K, Matsushita M, Okazaki K. Pancreatic cancer associated with autoimmune pancreatitis in remission. Intern Med 2008,47:151-5. Go to original source... Go to PubMed...
  29. Park DJ, Kim MH, Chari ST. Recent advances in autoimmune pancreatitis. Gut 2009,58:1680-9. Go to original source... Go to PubMed...
  30. Erkelens GW, Vleggaar FP, Lesterhuis W, van Buuren HR, van der Werf SD. Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet1999,354:43-4. Go to original source... Go to PubMed...
  31. Tanaka S, Kobayashi T, Nakanishi K, Okubo M, Murase T, Hashimoto M. Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis. Lancet 2000,356:910-1. Go to original source... Go to PubMed...
  32. Kountouras J, Zavos C, Gavalas E, Tzilves D. Challenge in the pathogenesis of autoimmune pancreatitis: potential role of Helicobacter pylori infection via molecular mimicry. Gasroenterology 2007,133:368-9. Go to original source... Go to PubMed...
  33. Ko SBH, Mizuno N, Yatabe Y, Yoshikawa T, Ishiguro H, Yamamoto A, Azuma S, Naruse S, Yamao K, Muallem S, Goto H. Corticosteroids correct abferant CFTR localization in the duct and regenerate acinar cells in autoimmune pancreatitis. Gastroenterology 2010,138,5:1988-96. Go to original source... Go to PubMed...
  34. Hart PA, Topazian MD, Witzig TE. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut Published Online First:30 Aug 2012, doi:10.1136/gutjnl-2012-302886/ Go to original source...
  35. Chari ST. Current concepts in the treatment of autoimmune pancreatitis. JOP 2007,8:1-3. Go to PubMed...
  36. Kamisawa T, Okamoto A. IgG - related sclerosing disease. World J Gastroenterol 2008,14:3948-55. Go to original source... Go to PubMed...
  37. Kamisawa T. IgG4-positive plasma cells specifically infiltrate various organs in autoimmune pancreatitis. Pancreas 2004,29:167-8. Go to original source... Go to PubMed...
  38. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K. A new clinicopathological entity of IgG4 - related autoimmune disease. J Gastroenterol 2003,38:982-4. Go to original source... Go to PubMed...
  39. Uomo G.: Autoimmune pancreatitis: An autonomous disease or a sigle entity in a complex puzzle of a multi-organ disorder? JOP (Online) 2010,5,11(2):191-2.
  40. Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, Takahashi H, Shinomura Y, Imai K, Saeki T, Azumi A, Nakada S, Sugiyama E, Matsui S, Origuchi T, Nishiyama S, Nishimori I, Nojima T, Yamada K, Kawano M, Zen Y, Kaneko M, Miyazaki K, Tsubota K, Eguchi K, Tomoda K, Sawaki T, Kawanami T, Tanaka M, Fukushima T, Sugai S, Umehara H. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009;68(8):1310-5. doi: 10.1136/ard.2008.089169 Go to original source... Go to PubMed...
  41. Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T. Histopathologic and clinical subtypes of autoimmune pancreatitis: The Honolulu Consensus Document. Pancreatology 2010,10:664-72. Go to original source... Go to PubMed...
  42. Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim MH, Klöppel G, Lerch MM, Löhr M, Notohara K, Okazaki K, Schneider A, Zhang L. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 2011,40(3):352-8. doi: 10.1097/MPA.0b013e3182142fd2. Go to original source... Go to PubMed...
  43. Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Recent advances in autoimmune pancreatitis type 1 and type 2. Gut 2013,62:1373-80. doi: 10.1136/gutjnl-2012-304224 Go to original source... Go to PubMed...
  44. Hart PA, Kamisawa T, Brugge WR, Chung JB, Culvert EL, Czako L, Frulloni L, Go WL, Gress TM, Myung-Hwan K, Kawa S, Lee KT, Lerch MM, Liao W, Lohr M, Okazaki K, Ryu JK, Schleinitz N, Shimizu K, Shimosegawa T, Soetikno R, Webster G, Yadav D, Zen Y, Chari ST. Long-term outcomes of autoimmune pacreatitis: a multicentgre, international analysis. Gut 2013;62(12):1771-6. doi: 10.1136/gutjnl-2012-303617 Go to original source... Go to PubMed...