Follicular helper T cells and follicular regulatory T cells in the immunopathology of primary Sjögren's syndrome

Journal of Leukocyte Biology: An Official Publication of the Reticuloendothelial Society

article

Weiqian Chen¹  Fan Yang²  Guanhua Xu¹  Jilin Ma³  Jin Lin¹ 

[1] Division of Rheumatology, First Affiliated Hospital, College of Medicine, Zhejiang University;State Key Laboratory of Infectious Diseases Diagnosis and Treatment, First Affiliated Hospital, College of Medicine, Zhejiang University;Division of Nephrology, Zhejiang Traditional Chinese Medicine and Western Medicine Hospital

关键词: primary Sjögren's syndrome;    Tfh cells;    Tfr cells;    CXCR5;    PD-1;    germinal center;   

DOI  :  10.1002/JLB.5MR1020-057RR

学科分类：生理学

来源: Federation of American Societies for Experimental Biology

【 摘 要 】

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease, characterized by lymphocytic infiltration into exocrine glands, which causes dry eyes, dry mouth, and systemic damage. Although the precise etiology of pSS is not clear yet, highly activated B cells, abundant anti-SSA/Ro, and anti-SSB/La autoantibodies are the hallmarks of this disease. Follicular helper T cells (Tfh), a subset of CD4 + T cells, with cell surface receptors PD-1 and CXCR5, express ICOS, transcription factor Bcl-6, and a cytokine IL-21. These cells help in the differentiation of B cells into plasma cells and stimulate the formation of germinal center (GC). Previous studies have demonstrated abundant Tfh cells in the peripheral blood and salivary glands (SGs) of the patients with pSS, correlated with extensive lymphocytic infiltration of the SGs and high disease activity scores. Patients with pSS who are treated with abatacept (CTLA-4 Ig) show fewer circulating Tfh cells, reduced expression of ICOS, and lower disease activity scores. Recently identified follicular regulatory T (Tfr) cells, a subset of regulatory T cells, control the function of Tfh cells and the GC reactions. Here, we summarize the observed alterations in Tfh and Tfr cell numbers, activation state, and circulating subset distribution in pSS. Our goal is to improve the understanding of the roles of Tfh and Tfr cells (surface marker expression, cytokine production, and transcription factors) in the pathogenesis of pSS, thus contributing to the identification of candidate therapeutic agents for this disease.

【 授权许可】

CC BY