Abstract
Systemic sclerosis is an autoimmune disease characterized by excessive dermal fibrosis with progression to internal organs, vascular impairment and immune dysregulation evidenced by the infiltration of inflammatory cells in affected tissues and the production of auto antibodies. While the pathogenesis remains unclear, several data highlight that T and B cells deregulation is implicated in the disease pathogenesis. Over the last decade, aberrant responses of circulating T follicular helper cells, a subset of CD4 T cells which are able to localise predominantly in the B cell follicles through a high level of chemokine receptor CXCR5 expression are described in pathogenesis of several autoimmune diseases and chronic graft-versus-host-disease. In the present review, we summarized the observed alteration of number and frequency of circulating T follicular helper cells in systemic sclerosis. We described their role in aberrant B cell activation and differentiation though interleukine-21 secretion. We also clarified T follicular helper-like cells involvement in fibrogenesis in both human and mouse model. Finally, because T follicular helper cells are involved in both fibrosis and autoimmune abnormalities in systemic sclerosis patients, we presented the different strategies could be used to target T follicular helper cells in systemic sclerosis, the therapeutic trials currently being carried out and the future perspectives from other auto-immune diseases and graft-versus-host-disease models.
Highlights
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by excessive skin fibrosis with progression to internal organs supported by activation of fibroblasts and excessive deposition of extra cellular matrix (ECM) [1]
Based on the extent of cutaneous fibrosis, two main forms of the disease have been identified, limited cutaneous SSc defined by skin fibrosis restricted to distal areas and diffuse cutaneous SSc associated with visceral fibrosis
Targeting TFH in systemic sclerosis: Perspectives from other auto‐immune diseases and GVHD models Because T follicular helper (Tfh) cells are involved in both fibrosis and autoimmune abnormalities in SSc patients, targeting Tfh cells represents an interesting therapeutic pathway
Summary
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by excessive skin fibrosis with progression to internal organs supported by activation of fibroblasts and excessive deposition of extra cellular matrix (ECM) [1]. In human cGVHD, cTfh cells seem to be decreased [21,22,23] but they express an activated phenotype and have a high capacity to promote B-cell immunoglobulin secretion and maturation [22]. In vitro Tfh cells co-cultured with autologous B cells from SSc patients enhanced plasmablast differentiation and induced high level of immunoglobulin production [24].
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