Abstract
Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltration and tissue destruction of exocrine glands such as salivary glands. Although the formation of ectopic lymphoid tissue in exocrine glands and overproduction of autoantibodies by autoreactive B cells highlight the critical involvement of B cells in disease development, the precise roles of various B cell subsets in pSS pathogenesis remain partially understood. Current studies have identified several novel B cell subsets with multiple functions in pSS, among which autoreactive age-associated B cells, and plasma cells with augmented autoantibody production contribute to the disease progression. In addition, tissue-resident Fc Receptor-Like 4 (FcRL4)+ B cell subset with enhanced pro-inflammatory cytokine production serves as a key driver in pSS patients with mucosa-associated lymphoid tissue (MALT)-lymphomas. Recently, regulatory B (Breg) cells with impaired immunosuppressive functions are found negatively correlated with T follicular helper (Tfh) cells in pSS patients. Further studies have revealed a pivotal role of Breg cells in constraining Tfh response in autoimmune pathogenesis. This review provides an overview of recent advances in the identification of pathogenic B cell subsets and Breg cells, as well as new development of B-cell targeted therapies in pSS patients.
Highlights
Primary Sjögren’s syndrome is a common systemic autoimmune disease, which mainly affects the lacrimal and salivary glands, resulting in dry eyes and dry mouth
Further studies on the comparison of Fc Receptor-Like 4 (FcRL4)+ B cells with chemokine receptor CCR5 expression profiles from the parotid glands of Primary Sjögren’s syndrome (pSS) patients provide a perspective for understanding their migratory ability to the corresponding chemokines CCL3 and CCL5 produced by ductal epithelial cells and their infiltration in the inflamed glands
The results showed that sixty percent of pSS patients achieved the primary endpoint whereas the disease activity index was significantly decreased at week 28
Summary
Primary Sjögren’s syndrome (pSS) is a common systemic autoimmune disease, which mainly affects the lacrimal and salivary glands, resulting in dry eyes and dry mouth. Growing evidence indicates that B cells play predominant roles in the pathogenesis of pSS patients with ectopic germinal center-like structures in the exocrine glands and systemic extra-glandular manifestations [5, 6]. Breg cells with different phenotypes have been reported to be involved in the development of pSS [13,14,15,16]. Breg cells exerted their regulatory functions by producing diverse regulatory cytokines and effector molecules, such as IL-10, IL-35 and Granzyme B (GrB). We discuss the multiple functions of B cell subsets in pSS development and emerging B cell-targeted therapies
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