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 articlepeer-review

66 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 - 2018 Feb 1


  • Germinal centre
  • IgG4-related disease
  • Interleukin-21
  • Interleukin-4
  • Pathogenesis
  • Plasmablast
  • Somatic hypermutation
  • T follicular helper cell
  • T lymphocyte

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)


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