Journal
RHEUMATOLOGY
Volume 57, Issue 2, Pages 236-245Publisher
OXFORD UNIV PRESS
DOI: 10.1093/rheumatology/kex171
Keywords
IgG4-related disease; pathogenesis; T lymphocyte; T follicular helper cell; germinal centre; plasmablast; somatic hypermutation; interleukin-4; interleukin-21
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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.
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