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An Overview

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IgG4-Related Disease

Abstract

Conferences focusing on IgG4-related disease started in 2007 in Japan comprising a small group of Japanese researchers, as a meeting on type 1 autoimmune pancreatitis (AIP) and the various types of extrapancreatic organ involvement. It raised the field to a higher status, and the attendees resolved to conduct annual meetings for the study of “IgG4-related disease (IgG4-RD).” Subsequently, intensive studies on IgG4-RD have been carried out worldwide. In this chapter, we focused on the establishment of the concept of IgG4-RD, with special reference to IgG4 as a subclass of IgG, conditions causing abnormal elevations of IgG4 or associated with IgG4, clinical picture of IgG4-RD, and mechanisms underlying its development.

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References

  1. Zen Y, Fujii T, Harada K, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology. 2007;45(6):1538–46.

    Article  PubMed  CAS  Google Scholar 

  2. Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811–2.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  4. 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  PubMed  CAS  Google Scholar 

  5. Ohara H, Nakazawa T, Sano H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31(3):232–7.

    Article  PubMed  Google Scholar 

  6. Hamano H, Arakura N, Muraki T, et al. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41(12):1197–205.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  8. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.

    Article  PubMed  Google Scholar 

  9. Delves PJ, Martin SJ, Burton DR, Roitt IM. Roitt’s essential immunology. 12th ed. Hoboken: Wiley; 2011. ISBN 97814051966833.

    Google Scholar 

  10. Muraki T, Hamano H, Ochi Y, et al. Autoimmune pancreatitis and complement activation system. Pancreas. 2006;32(1):16–21.

    Article  PubMed  Google Scholar 

  11. Saeki T, Nishi S, Imai N, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2010;78(10):1016–23.

    Article  PubMed  CAS  Google Scholar 

  12. Kawano M, Saeki T, Nakashima H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15(5):615–26.

    Article  PubMed  Google Scholar 

  13. Cornell LD, Chicano SL, Deshpande V, et al. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease. Am J Surg Pathol. 2007;31(10):1586–97.

    Article  PubMed  Google Scholar 

  14. Raissian Y, Nasr SH, Larsen CP, et al. Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 2011 Jul;22(7):1343–52.

    Article  PubMed  Google Scholar 

  15. Yamaguchi Y, Kanetsuna Y, Honda K, et al. Characteristic tubulointerstitial nephritis in IgG4-related disease. Hum Pathol. 2011;43(4):536–49.

    Article  PubMed  Google Scholar 

  16. van der Neut KM, Schuurman J, Losen M, et al. Anti-inflammatory activity of human IgG4 antibodies by dynamic Fab arm exchange. Science. 2007;317(5844):1554–7.

    Article  Google Scholar 

  17. Rispens T, Ooievaar-De Heer P, Vermeulen E, et al. Human IgG4 binds to IgG4 and conformationally altered IgG1 via Fc-Fc interactions. J Immunol. 2009;182(7):4275–81.

    Article  PubMed  CAS  Google Scholar 

  18. Kawa S, Kitahara K, Hamano H, et al. A novel immunoglobulin-immunoglobulin interaction in autoimmunity. PLoS One. 2008;3(2):e1637.

    Article  PubMed  Google Scholar 

  19. Shakib F, Stanworth DR, Drew R, et al. A quantitative study of the distribution of IgG sub-classes in a group of normal human sera. J Immunol Methods. 1975;8(1–2):17–28.

    Article  PubMed  CAS  Google Scholar 

  20. Fernandez-Flores A. The role of IgG4 in cutaneous pathology. Rom J Morphol Embryol. 2012;53(2):221–31.

    PubMed  CAS  Google Scholar 

  21. Manso EC, Morato-Castro FF, Yee CJ, et al. Honeybee venom specific IgG subclass antibodies in Brazilian beekeepers and in patients allergic to bee stings. J Investig Allergol Clin Immunol. 1998;8(1):46–51.

    PubMed  CAS  Google Scholar 

  22. Ottesen EA, Skvaril F, Tripathy SP, et al. Prominence of IgG4 in the IgG antibody response to human filariasis. J Immunol. 1985;134(4):2707–12.

    PubMed  CAS  Google Scholar 

  23. Iskander R, Das PK, Aalberse RC. IgG4 antibodies in Egyptian patients with schistosomiasis. Int Arch Allergy Appl Immunol. 1981;66(2):200–7.

    Article  PubMed  CAS  Google Scholar 

  24. Klooster R, Plomp JJ, Huijbers MG, et al. Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice. Brain. 2012;135(Pt 4):1081–101.

    Article  PubMed  Google Scholar 

  25. Beck Jr LH, Salant DJ. Membranous nephropathy: recent travels and new roads ahead. Kidney Int. 2010;77(9):765–70.

    Article  PubMed  CAS  Google Scholar 

  26. Ferrari S, Mudde GC, Rieger M, et al. IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2009;7(10):1703–10.

    Article  PubMed  CAS  Google Scholar 

  27. Masaki Y, Dong L, Kurose N, et al. 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.

    Article  PubMed  CAS  Google Scholar 

  28. Okazaki K, Chiba T. Autoimmune related pancreatitis. Gut. 2002;51(1):1–4.

    Article  PubMed  CAS  Google Scholar 

  29. Kawa S, Hamano H. Clinical features of autoimmune pancreatitis. J Gastroenterol. 2007;42 Suppl 18:9–14.

    Article  PubMed  Google Scholar 

  30. Kamisawa T, Takuma K, Egawa N, et al. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol. 2010;7(7):401–9.

    Article  PubMed  CAS  Google Scholar 

  31. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30.

    Article  PubMed  CAS  Google Scholar 

  32. Sato Y, Kojima M, Takata K, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease. Mod Pathol. 2009;22(4):589–99.

    Article  PubMed  CAS  Google Scholar 

  33. Matsubayashi H, Uesaka K, Kanemoto H, et al. Soluble IL-2 receptor, a new marker for autoimmune pancreatitis. Pancreas. 2012;41(3):493–6.

    Article  PubMed  Google Scholar 

  34. Masaki Y, Kurose N, Yamamoto M, et al. Cutoff values of serum IgG4 and histopathological IgG4+ plasma cells for diagnosis of patients with IgG4-related disease. Int J Rheumatol. 2012;2012:580814.

    PubMed  Google Scholar 

  35. Yamamoto M, Tabeya T, Naishiro Y, et al. Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases. Mod Rheumatol. 2011;22(3):419–25.

    Article  PubMed  Google Scholar 

  36. Strehl JD, Hartmann A, Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64(3):237–43.

    Article  PubMed  Google Scholar 

  37. Houghton DC, Troxell ML. An abundance of IgG4+ plasma cells is not specific for IgG4-related tubulointerstitial nephritis. Mod Pathol. 2011;24(11):1480–7.

    Article  PubMed  CAS  Google Scholar 

  38. Watanabe T, Yamashita K, Fujikawa S, et al. Involvement of activation of toll-like receptors and nucleotide-binding oligomerization domain-like receptors in enhanced IgG4 responses in autoimmune pancreatitis. Arthritis Rheum. 2012;64(3):914–24.

    Article  PubMed  CAS  Google Scholar 

  39. Watanabe T, Yamashita K, Sakurai T, et al. Toll-like receptor activation in basophils contributes to the development of IgG4-related disease. J Gastroenterol. 2013;48(2):247–53.

    Article  PubMed  CAS  Google Scholar 

  40. Kiyama K, Kawabata D, Hosono Y, et al. Serum BAFF and APRIL levels in patients with IgG4-related disease and their clinical significance. Arthritis Res Ther. 2012;14(2):R86.

    Article  PubMed  CAS  Google Scholar 

  41. Okazaki K. Clinical relevance of autoimmune-related pancreatitis. Best Pract Res Clin Gastroenterol. 2002;16(3):365–78.

    Article  PubMed  CAS  Google Scholar 

  42. Guarneri F, Guarneri C, Benvenga S. Helicobacter pylori and autoimmune pancreatitis: role of carbonic anhydrase via molecular mimicry? J Cell Mol Med. 2005;9(3):741–4.

    Article  PubMed  CAS  Google Scholar 

  43. Kawa S, Ota M, Yoshizawa K, et al. HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology. 2002;122(5):1264–9.

    Article  PubMed  Google Scholar 

  44. Freitag TL, Cham C, Sung HH, et al. Human risk allele HLA-DRB1*0405 predisposes class II transgenic Ab0 NOD mice to autoimmune pancreatitis. Gastroenterology. 2010;139(1):281–91.

    Article  PubMed  CAS  Google Scholar 

  45. Tanaka A, Moriyama M, Nakashima H, et al. Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. Arthritis Rheum. 2012;64(1):254–63.

    Article  PubMed  CAS  Google Scholar 

  46. Maehara T, Moriyama M, Nakashima H, et al. Interleukin-21 contributes to germinal centre formation and immunoglobulin G4 production in IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz's disease. Ann Rheum Dis. 2012;71(12):2011–20.

    Article  PubMed  CAS  Google Scholar 

  47. Yamamoto M, Takahashi H, Tabeya T, et al. Risk of malignancies in IgG4-related disease. Mod Rheumatol. 2011;22(3):414–8.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Center for Health, Safety, and Environmental Management, Shinshu University, 3-1-1, Asahi, Matsumoto City, 390-8621, Japan

    Shigeyuki Kawa

  2. Division of Rheumatology, Kanazawa University Hospital, Takara-machi, Kanazawa, Ishikawa, 920-8641, Japan

    Mitsuhiro Kawano

Authors
  1. Shigeyuki Kawa
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  2. Mitsuhiro Kawano
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Corresponding author

Correspondence to Shigeyuki Kawa .

Editor information

Editors and Affiliations

  1. Department of Hematology and Immunology, Kanazawa Medical University, Ishikawa, Japan

    Hisanori Umehara

  2. The 3rd Department of Internal Medicine Div of Gastroenterology and Hepatology, Kansai Medical Univ Hirakata Hospital, Osaka, Osaka, Japan

    Kazuichi Okazaki

  3. Massachusetts General Hospital, Boston, Massachusetts, USA

    John H. Stone

  4. Shinshu University Center for Health Safety and Environment, Matsumoto, Nagano, Japan

    Shigeyuki Kawa

  5. Kanazawa University, Kanazawa, Ishikawa, Japan

    Mitsuhiro Kawano

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© 2014 Springer Japan

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Kawa, S., Kawano, M. (2014). An Overview. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_1

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  • DOI: https://doi.org/10.1007/978-4-431-54228-5_1

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54227-8

  • Online ISBN: 978-4-431-54228-5

  • eBook Packages: MedicineMedicine (R0)

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