Imbalance between T helper 17 and regulatory T cell subsets plays a significant role in the pathogenesis of systemic sclerosis.

Systemic sclerosis (SSc) is a rare autoimmune disease that is characterized by fibrosis, inflammation, and vasculopathy of the skin and internal organs. The etiopathogenesis of SSc remains unclear. However, the pivotal role of T lymphocytes with an aberrant immune response in SSc is well established. Among T cells, IL-17-producing helper T (Th17) cell and regulatory T (Treg) cell subsets have recently been found to play crucial roles in SSc pathogenesis. Generally speaking, Th17 cell subsets up-regulate inflammation, fibrosis, and autoimmunity, which are present in SSc, while Treg cell subsets have an immunosuppressive function and resist the immunological performance of Th17 cells. Up-to-date evidence has pointed out that the imbalance and abnormal functions of Th17/Treg cells may contribute to SSc. Therefore, this review aims to summarize the current understanding of the vital cytokines and signaling pathways that are involved in Th17/Treg differentiation and functions, and their roles in the pathogenesis of SSc, thus providing novel insights about targeting the Th17/Treg balance as a potential therapy for SSc treatment in the near future.