Gastrointestinal Involvement in IgG4-Related Disease

  • Mitsuru SugimotoEmail author
  • Erina Suzuki
  • Kazuhiro Tasaki
  • Yuko Hashimoto
  • Hiromasa Ohira


IgG4-related disease (IgG4-RD) is characterized by increased serum IgG4 and swelling of several organs or nodules by infiltration of IgG4-positive plasma cells and lymphocytes, as well as fibrosis observed throughout the body. In the gastrointestinal area, autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC) are primarily observed. In 2010, the International Consensus Diagnostic Criteria (ICDC) for AIP were proposed, and in 2012, clinical diagnostic criteria for IgG4-SC were proposed. Nevertheless, the aetiology of IgG4-RD remains unknown, and histological diagnosis of gastrointestinal IgG4-RD remains difficult. Differentiation between gastrointestinal IgG4-RD and malignant pancreaticobiliary diseases is difficult. Steroids have become the established therapy for IgG4-RD; however, predictive relapse factors are controversial. In this chapter, we introduce the history, diagnosis and treatment of gastrointestinal IgG4-RD, as well as several challenges to ameliorating the difficulties mentioned above.


IgG4-related disease Autoimmune pancreatitis IgG4-related sclerosing cholangitis 


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Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Mitsuru Sugimoto
    • 1
    Email author
  • Erina Suzuki
    • 2
  • Kazuhiro Tasaki
    • 2
  • Yuko Hashimoto
    • 2
  • Hiromasa Ohira
    • 1
  1. 1.Department of GastroenterologyFukushima Medical University School of MedicineFukushimaJapan
  2. 2.Department of Diagnostic PathologyFukushima Medical University School of MedicineFukushimaJapan

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