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Pathology

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Autoimmune Pancreatitis

Abstract

Before the entity of autoimmune pancreatitis (AIP) was proposed clinically [1], several disease entities that are now regarded as equivalent to AIP were reported from the pathological standpoint: chronic inflammatory sclerosis [2], lymphoplasmacytic sclerosing pancreatitis (LPSP) [3], and nonalcoholic duct-destructive chronic pancreatitis [4]. Pathologists later recognized that there were at least two different concepts in what had been considered AIP, and these were named LPSP/AIP without granulocytic epithelial lesion (GEL) and idiopathic duct-centric pancreatitis (IDCP)/AIP with GEL [5, 6]. The discovery of elevated serum IgG4 in patients with AIP by Hamano and colleagues motivated further research into AIP [7], and LPSP is now regarded as the pancreatic manifestation of IgG4-related disease (IgG4-RD) in the present consensus [8–10]. In addition, the subclassification of AIP was proposed based on its different demographics and clinical features [11–13], and LPSP and IDCP were designated as type 1 and type 2 AIP, respectively [14]. In fact, the histological features of type 1 and type 2 AIP are distinct, and pathologists can distinguish them with hematoxylin and eosin (HE) stain alone without the aid of IgG4 immunostaining, which was validated by an international interobserver study [15].

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References

  1. 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(7):1561–8.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  3. 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(4):387–95.

    Article  CAS  PubMed  Google Scholar 

  4. Ectors N, Maillet B, Aerts R, Geboes K, Donner A, Borchard F, Lankisch P, Stolte M, Luttges J, Kremer B, Kloppel G. Non-alcoholic duct destructive chronic pancreatitis. Gut. 1997;41(2):263–8.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  5. 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(8):1119–27.

    Article  PubMed  Google Scholar 

  6. Zamboni G, Luttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Kloppel 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(6):552–63.

    Article  PubMed  Google Scholar 

  7. 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(10):732–8.

    Article  CAS  PubMed  Google Scholar 

  8. 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(5):683–7.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, Hamano H, Kamisawa T, Shimosegawa T, Shimatsu A, Nakamura S, Ito T, Notohara K, Sumida T, Tanaka Y, Mimori T, Chiba T, Mishima M, Hibi T, Tsubouchi H, Inui K, Ohara H. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30.

    Article  CAS  PubMed  Google Scholar 

  10. Stone JH, Khosroshahi A, Deshpande V, Chan JK, Heathcote JG, Aalberse R, Azumi A, Bloch DB, Brugge WR, Carruthers MN, Cheuk W, Cornell L, Castillo CF, Ferry JA, Forcione D, Kloppel G, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Masaki Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani D, Sato Y, Smyrk T, Stone JR, Takahira M, Umehara H, Webster G, Yamamoto M, Yi E, Yoshino T, Zamboni G, Zen Y, Chari S. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum. 2012;64(10):3061–7.

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  12. Kamisawa T, Chari ST, Giday SA, Kim MH, Chung JB, Lee KT, Werner J, Bergmann F, Lerch MM, Mayerle J, Pickartz T, Lohr M, Schneider A, Frulloni L, Webster GJ, Reddy DN, Liao WC, Wang HP, Okazaki K, Shimosegawa T, Kloeppel G, Go VL. Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas. 2011;40(6):809–14.

    Article  CAS  PubMed  Google Scholar 

  13. Song TJ, Kim JH, Kim MH, Jang JW, Park do H, Lee SS, Seo DW, Lee SK, Yu E. Comparison of clinical findings between histologically confirmed type 1 and type 2 autoimmune pancreatitis. J Gastroenterol Hepatol. 2012;27(4):700–8.

    Article  CAS  PubMed  Google Scholar 

  14. Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T. Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39(5):549–54.

    Article  CAS  PubMed  Google Scholar 

  15. Zhang L, Chari S, Smyrk TC, Deshpande V, Kloppel G, Kojima M, Liu X, Longnecker DS, Mino-Kenudson M, Notohara K, Rodriguez-Justo M, Srivastava A, Zamboni G, Zen Y. Autoimmune pancreatitis (AIP) type 1 and type 2: an international consensus study on histopathologic diagnostic criteria. Pancreas. 2011;40(8):1172–9.

    Article  CAS  PubMed  Google Scholar 

  16. 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-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol. 2004;28(9):1193–203.

    Article  PubMed  Google Scholar 

  17. Kitagawa S, Zen Y, Harada K, Sasaki M, Sato Y, Minato H, Watanabe K, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Tsuneyama K, Saito K, Haratake J, Takagawa K, Nakanuma Y. Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner’s tumor). Am J Surg Pathol. 2005;29(6):783–91.

    Article  PubMed  Google Scholar 

  18. Notohara K, Wani Y, Fujisawa M. Pathologic findings of autoimmune pancreatitis and IgG4-related disease. Curr Immunol Rev. 2011;7(2):212–20.

    Article  CAS  Google Scholar 

  19. Irie H, Honda H, Baba S, Kuroiwa T, Yoshimitsu K, Tajima T, Jimi M, Sumii T, Masuda K. Autoimmune pancreatitis: CT and MR characteristics. AJR Am J Roentgenol. 1998;170(5):1323–7.

    Article  CAS  PubMed  Google Scholar 

  20. Miyabe K, Notohara K, Nakazawa T, Hayashi K, Naitoh I, Okumura F, Shimizu S, Yoshida M, Yamashita H, Takahashi S, Ohara H, Joh T. Histological evaluation of obliterative phlebitis for the diagnosis of autoimmune pancreatitis. J Gastroenterol. 2013;49(4):715–26.

    Article  PubMed  Google Scholar 

  21. Kasashima S, Zen Y, Kawashima A, Konishi K, Sasaki H, Endo M, Matsumoto Y, Kawakami K, Kasashima F, Moriya M, Kimura K, Ohtake H, Nakanuma Y. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32(2):197–204.

    Article  PubMed  Google Scholar 

  22. 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(1):23–8.

    Article  CAS  PubMed  Google Scholar 

  23. Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim MH, Kloppel G, Lerch MM, Lohr 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.

    Article  PubMed  Google Scholar 

  24. Shimosegawa T. 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(8):1341–2.

    Article  PubMed  Google Scholar 

  25. Dhall D, Suriawinata AA, Tang LH, Shia J, Klimstra DS. Use of immunohistochemistry for IgG4 in the distinction of autoimmune pancreatitis from peritumoral pancreatitis. Hum Pathol. 2010;41(5):643–52.

    Article  CAS  PubMed  Google Scholar 

  26. Suda K, Takase M, Fukumura Y, Kashiwagi S. Pathology of autoimmune pancreatitis and tumor-forming pancreatitis. J Gastroenterol. 2007;42 Suppl 18:22–7.

    Article  PubMed  Google Scholar 

  27. Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, Kloppel G, Heathcote JG, Khosroshahi A, Ferry JA, Aalberse RC, Bloch DB, Brugge WR, Bateman AC, Carruthers MN, Chari ST, Cheuk W, Cornell LD, Fernandez-Del Castillo C, Forcione DG, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Lauwers GY, Masaki Y, Nakanuma Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani DV, Smyrk TC, Stone JR, Takahira M, Webster GJ, Yamamoto M, Zamboni G, Umehara H, Stone JH. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.

    Article  PubMed  Google Scholar 

  28. Motosugi U, Ichikawa T, Yamaguchi H, Nakazawa T, Katoh R, Itakura J, Fujii H, Sato T, Araki T, Shimizu M. Small invasive ductal adenocarcinoma of the pancreas associated with lymphoplasmacytic sclerosing pancreatitis. Pathol Int. 2009;59(10):744–7.

    Article  PubMed  Google Scholar 

  29. Witkiewicz AK, Kennedy EP, Kennyon L, Yeo CJ, Hruban RH. Synchronous autoimmune pancreatitis and infiltrating pancreatic ductal adenocarcinoma: case report and review of the literature. Hum Pathol. 2008;39(10):1548–51.

    Article  PubMed  Google Scholar 

  30. Oh HC, Kim JG, Kim JW, Lee KS, Kim MK, Chi KC, Kim YS, Kim KH. Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis. Intern Med. 2008;47(23):2025–8.

    Article  PubMed  Google Scholar 

  31. Ohtani H, Ishida H, Ito Y, Yamaguchi T, Koizumi M. Autoimmune pancreatitis and biliary intraepithelial neoplasia of the common bile duct: a case with diagnostically challenging but pathogenetically significant association. Pathol Int. 2011;61(8):481–5.

    Article  PubMed  Google Scholar 

  32. Naitoh I, Nakazawa T, Notohara K, Miyabe K, Hayashi K, Shimizu S, Kondo H, Yoshida M, Yamashita H, Umemura S, Ohara H, Joh T. Intraductal papillary mucinous neoplasm associated with autoimmune pancreatitis. Pancreas. 2013;42(3):552–4.

    Article  PubMed  Google Scholar 

  33. Urata T, Naito Y, Izumi Y, Takekuma Y, Yokomizo H, Nagamine M, Fukuda S, Notohara K, Hifumi M. Localized type 1 autoimmune pancreatitis superimposed upon preexisting intraductal papillary mucinous neoplasms. World J Gastroenterol. 2013;19(47):9127–32.

    Article  PubMed Central  PubMed  Google Scholar 

  34. Tabata T, Kamisawa T, Hara S, Kuruma S, Chiba K, Kuwata G, Fujiwara T, Egashira H, Koizumi S, Endo Y, Koizumi K, Fujiwara J, Arakawa T, Momma K, Horiguchi S, Hishima T, Kurata M, Honda G, Kloppel G. Intraductal papillary mucinous neoplasm of the pancreas and IgG4-related disease: a coincidental association. Pancreatology. 2013;13(4):379–83.

    Article  CAS  PubMed  Google Scholar 

  35. Fukui T, Mitsuyama T, Takaoka M, Uchida K, Matsushita M, Okazaki K. Pancreatic cancer associated with autoimmune pancreatitis in remission. Intern Med. 2008;47(3):151–5.

    Article  PubMed  Google Scholar 

  36. Shiokawa M, Kodama Y, Yoshimura K, Kawanami C, Mimura J, Yamashita Y, Asada M, Kikuyama M, Okabe Y, Inokuma T, Ohana M, Kokuryu H, Takeda K, Tsuji Y, Minami R, Sakuma Y, Kuriyama K, Ota Y, Tanabe W, Maruno T, Kurita A, Sawai Y, Uza N, Watanabe T, Haga H, Chiba T. Risk of cancer in patients with autoimmune pancreatitis. Am J Gastroenterol. 2013;108(4):610–7.

    Article  CAS  PubMed  Google Scholar 

  37. 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(3):594–602.

    Article  PubMed  Google Scholar 

  38. Levy MJ, Reddy RP, Wiersema MJ, Smyrk TC, Clain JE, Harewood GC, Pearson RK, Rajan E, Topazian MD, Yusuf TE, Chari ST, Petersen BT. 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 

  39. Mizuno N, Bhatia V, Hosoda W, Sawaki A, Hoki N, Hara K, Takagi T, Ko SB, Yatabe Y, Goto H, Yamao K. Histological diagnosis of autoimmune pancreatitis using EUS-guided trucut biopsy: a comparison study with EUS-FNA. J Gastroenterol. 2009;44(7):742–50.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgment

This work was supported in part by the Health and Labour Sciences Research Grants (Intractable Diseases) from the Japanese Ministry of Health, Labour, and Welfare.

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Authors and Affiliations

  1. Department of Anatomic Pathology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, 710-8602, Japan

    Kenji Notohara MD, PhD

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  1. Kenji Notohara MD, PhD
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Correspondence to Kenji Notohara MD, PhD .

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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

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Notohara, K. (2015). Pathology. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_4

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  • DOI: https://doi.org/10.1007/978-3-642-55086-7_4

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