Follicular helper T cells in the pathogenesis of IgG4-related disease

Mitsuhiro Akiyama, Katsuya Suzuki, Hidekata Yasuoka, Yuko Kaneko, Kunihiro Yamaoka, Tsutomu Takeuchi

Research output: Contribution to journalReview article

28 Citations (Scopus)


IgG4-related disease (IgG4-RD) is a recently recognized disease entity characterized by high serum IgG4 concentrations and infiltration of IgG4+ plasma cells with hyperplastic ectopic germinal centres at affected sites. Although the underlying immune mechanism of this disease remains unclear, T cells are abundantly present at affected sites and key players in IgG4-RD pathogenesis. The role of T cell subsets has been investigated thoroughly in this disease. Recent advances in this field have clarified the importance of T follicular helper cells. In this review, we describe the role of T follicular helper cells in the disease process of IgG4-RD, in particular, for IgG4 class-switching, plasmablast and plasma cell differentiation, and germinal centre formation.

Original languageEnglish
Article numberkex171
Pages (from-to)236-245
Number of pages10
JournalRheumatology (United Kingdom)
Issue number2
Publication statusPublished - 01-02-2018
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Rheumatology
  • Pharmacology (medical)

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    Akiyama, M., Suzuki, K., Yasuoka, H., Kaneko, Y., Yamaoka, K., & Takeuchi, T. (2018). Follicular helper T cells in the pathogenesis of IgG4-related disease. Rheumatology (United Kingdom), 57(2), 236-245. [kex171].