Skip to main content
SpringerLink
Log in

IgG4-Related Gastrointestinal Lesion

  • Chapter
  • First Online:
Autoimmune Pancreatitis

  • 1096 Accesses

Abstract

IgG4-related disease has recently been recognized as a systemic condition that is characterized by increased serum IgG4 levels and responsiveness to steroid therapy. Patients with IgG4-RD show organ enlargement or nodular lesions with abundant lymphocyte and IgG4-positive plasma cell infiltration and fibrosis, and they frequently present both clinically and radiologically with findings suggestive of malignancy, which may result in unnecessary resection [1–3]. According to the comprehensive clinical diagnostic criteria for IgG4-RD [4], the diagnosis of IgG4-RD is made in the presence of a characteristic diffuse/localized swelling or mass in one or more organs with increased serum IgG4 levels or histological findings of abundant IgG4-positive plasma cell and lymphocyte infiltration associated with fibrosis.

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. Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.

    Article  CAS  PubMed  Google Scholar 

  2. Kamisawa T, Takuma K, Egawa N, Tsuruta K, Sasaki T. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol. 2010;7:401–9.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  6. Kamisawa T, Egawa N, Nakajima H, et al. Gastrointestinal findings in Patients with autoimmune pancreatitis. Endoscopy. 2005;37:1127–30.

    Article  CAS  PubMed  Google Scholar 

  7. Deheragoda MG, Church NI, Rodriguez-Justo M, et al. The use of immunoglobulin g4 immunostaining in diagnosing pancreatic and extrapancreatic involvement in autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2007;5:1229–34.

    Article  PubMed  Google Scholar 

  8. 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 

  9. Sepehr A, Mino-Kenudson M, Ogawa F, Brugge WR, Deshpande V, Lauwers GY. IgG4+ to IgG + plasma cells ratio of ampulla can help differentiate autoimmune pancreatitis from other “mass forming” pancreatic lesions. Am J Surg Pathol. 2008;32:1770–9.

    Article  PubMed  Google Scholar 

  10. Hirano K, Fukushima N, Tada M, et al. Diagnostic utility of biopsy specimens for autoimmune pancreatitis. J Gastroenterol. 2009;44:765–73.

    Article  CAS  PubMed  Google Scholar 

  11. Kuwata G, Kamisawa T, Koizumi K, et al. Ulcerative colitis and immunoglobulin g4. Gut Liver. 2014;8:29–34.

    Article  PubMed Central  PubMed  Google Scholar 

  12. Lopes J, Hochwald SN, Lancia N, Dixon LR, Ben-David K. Autoimmune esophagitis: IgG4-related tumors of the esophagus. J Gastrointest Surg. 2010;14:1031–4.

    Article  PubMed  Google Scholar 

  13. Lee H, Joo M, Song TJ, et al. IgG4-related sclerosing esophagitis: a case report. Gastrointest Endosc. 2011;73:834–7.

    Article  PubMed  Google Scholar 

  14. Na KY, Sung JY, Jang JY, et al. Gastric nodular lesion caused by IgG4-related disease. Pathol Int. 2012;62:716–8.

    Article  PubMed  Google Scholar 

  15. Bateman AC, Sommerlad M, Underwood TJ. Chronic gastric ulceration: a novel manifestation of IgG4-related disease? J Clin Pathol. 2012;65:569–70.

    Article  PubMed  Google Scholar 

  16. Rollins KE, Mehta SP, O’Donovan M, Safranek PM. Gastric IgG4-related autoimmune fibrosclerosing pseudotumor: a novel location. ISRN Gastroenterol. 2011;2011:873087.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Kaji R, Okabe Y, Ishida Y, et al. Autoimmune pancreatitis presenting with IgG4-positive multiple gastric polyps. Gastrointest Endosc. 2010;71:420–2.

    Article  PubMed  Google Scholar 

  18. Kim do H, Kim J, Park do H, et al. Immunoglobulin G4-related inflammatory pseudotumor of the stomach. Gastrointest Endosc. 2012;76:451–2.

    Article  PubMed  Google Scholar 

  19. Fujita T, Ando T, Sakakibara M, Hosoda W, Goto H. Refractory gastric ulcer with abundant IgG4-positive plasma cell infiltration. World J Gastroenterol. 2010;16:2183–6.

    Article  PubMed Central  PubMed  Google Scholar 

  20. Chetty R, Serra S, Gauchotte G, Markl B, Agaimy A. Sclerosing nodular lesions of the gastrointestinal tract containing large numbers of IgG4 plasma cells. Pathology. 2011;43:31–5.

    Article  PubMed  Google Scholar 

  21. Kamisawa T, Takuma K, Tabata T, et al. Serum IgG4-negative autoimmune pancreatitis. J Gastroenterol. 2011;46:108–16.

    Article  CAS  PubMed  Google Scholar 

  22. Kamisawa T, Nakajima H, Egawa N, Hayashi Y, Funata N. Autoimmune pancreatitis can be confirmed with gastroscopy. Dig Dis Sci. 2004;49:155–6.

    Article  PubMed  Google Scholar 

  23. Hisa T, Ohkubo H, Shiozawa S, Ishigame H, Furutake M, Takamatsu M. Lymphoplasmacytic granuloma localized to the ampulla of Vater: an ampullary lesion of IgG4-related systemic disease? Gastrointest Endosc. 2008;68:1229–32.

    Article  PubMed  Google Scholar 

  24. Kubota K, Iida H, Fujisawa T, et al. Clinical significance of swollen duodenal papilla in autoimmune pancreatitis. Pancreas. 2007;35:e51–60.

    Article  PubMed  Google Scholar 

  25. Kim MH, Moon SH, Kamisawa T. Major duodenal papilla in autoimmune pancreatitis. Dig Surg. 2010;27:110–4.

    Article  PubMed  Google Scholar 

  26. Unno H, Saegusa H, Fukushima M, Hamano H. Usefulness of endoscopic observation of the main duodenal papilla in the diagnosis of sclerosing pancreatitis. Gastrointest Endosc. 2002;56:880–4.

    Article  PubMed  Google Scholar 

  27. Kamisawa T, Anjiki H, Egawa N. Disappearance of an ampullary pseudotumor after steroid therapy for autoimmune pancreatitis. Gastrointest Endosc. 2010;71:847–8.

    Article  PubMed  Google Scholar 

  28. Kamisawa T, Tu Y, Nakajima H, Egawa N, Tsuruta K, Okamoto A. Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis: a prospective study using IgG4-immunostaining. World J Gastroenterol. 2006;12:2031–3.

    PubMed Central  PubMed  Google Scholar 

  29. Moon SH, Kim MH, Park do H, et al. IgG4 immunostaining of duodenal papillary biopsy specimens may be useful for supporting a diagnosis of autoimmune pancreatitis. Gastrointest Endosc. 2010;71:960–6.

    Article  PubMed  Google Scholar 

  30. Matsui H, Watanabe T, Ueno K, et al. Colonic polyposis associated with autoimmune pancreatitis. Pancreas. 2009;38:840–2.

    Article  PubMed  Google Scholar 

  31. Ueno K, Watanabe T, Kawata Y, et al. IgG4-related autoimmune pancreatitis involving the colonic mucosa. Eur J Gastroenterol Hepatol. 2008;20:1118–21.

    Article  PubMed  Google Scholar 

  32. Akitake R, Watanabe T, Zaima C, et al. Possible involvement of T helper type 2 responses to Toll-like receptor ligands in IgG4-related sclerosing disease. Gut. 2010;59:542–5.

    Article  CAS  PubMed  Google Scholar 

  33. 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  CAS  PubMed  Google Scholar 

  34. Sepehr A, Lauwers GY. Gastrointestinal evidence of autoimmune pancreatitis: a rare manifestation. Histopathology. 2008;53:358–9.

    Article  CAS  PubMed  Google Scholar 

  35. Ravi K, Chari ST, Vege SS, Sandborn WJ, Smyrk TC, Loftus Jr EV. Inflammatory bowel disease in the setting of autoimmune pancreatitis. Inflamm Bowel Dis. 2009;15:1326–30.

    Article  PubMed  Google Scholar 

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

    Article  PubMed Central  PubMed  Google Scholar 

  37. Rebours V, Le Baleur Y, Cazals-Hatem D, et al. Immunoglobulin G4 immunostaining of gastric, duodenal, or colonic biopsies is not helpful for the diagnosis of autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2012;10:91–4.

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  39. Kamisawa T, Tabata T, Kuwata G, Koizumi K. Extraintestinal manifestations of inflammatory bowel disease: autoimmune pancreatitis and other IgG4-related conditions. In: Baumgart DC, editor. Crohn’s disease and ulcerative colitis. New York: Springer; 2012. p. 601–10.

    Chapter  Google Scholar 

  40. Kuroda Y, Fujioka M, Kurosawa K, Ohashi K. IgG4-related inflammatory pseudotumor of the ileal conduit. Pathol Int. 2011;61:47–8.

    Article  PubMed  Google Scholar 

  41. Wong DD, Pillai SR, Priyanthi Kumarasinghe M, et al. IgG4-related sclerosing disease of the small bowel presenting as necrotizing mesenteric arteritis and a solitary jejunal ulcer. Am J Surg Pathol. 2012;36:929–34.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  43. Zen Y, Harada K, Sasaki M, et al. 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;28:1193–203.

    Article  PubMed  Google Scholar 

  44. Zen Y, Kitagawa S, Minato H, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36:710–7.

    Article  CAS  PubMed  Google Scholar 

  45. Tsuboi H, Inokuma S, Setoguchi K, et al. Inflammatory pseudotumors in multiple organs associated with elevated serum IgG4 level: recovery by only a small replacement dose of steroid. Intern Med. 2008;47:1139–42.

    Article  PubMed  Google Scholar 

Download references

Acknowledgment

This study was partially supported by the Intractable Disease, supported by the Ministry of Health, Labour, and Welfare of Japan.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan

    Satomi Koizumi MD, Terumi Kamisawa MD, PhD & Sawako Kuruma MD

Authors
  1. Satomi Koizumi MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Terumi Kamisawa MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sawako Kuruma MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Terumi Kamisawa MD, PhD .

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

Koizumi, S., Kamisawa, T., Kuruma, S. (2015). IgG4-Related Gastrointestinal Lesion. In: Kamisawa, T., Chung, J. (eds) Autoimmune Pancreatitis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55086-7_21

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-55086-7_21

  • 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