Figure 1.

The SSc-specific common pathologic cascade across multiple organs. The accumulation of predisposing factors through the interaction of genetic factors and environmental influences induces the phenotypical alterations of immune cells, vascular cells and interstitial fibroblasts. Vascular injury due to autoimmune attacks and unknown environmental influences triggers the SSc-specific common pathologic cascade across multiple organs, starting with vascular activation and vascular structural abnormalities, and subsequently resulting in chronic inflammation and the related activation of interstitial fibroblasts derived from various sources. pDCs, plasmacytoid dendritic cells; DCs, dendritic cells; TGF-β, transforming growth factor-β; Th, T helper; EndoMT, endothelial-to-mesenchymal transition; EMT, epithelial-to-mesenchymal transition; AMT, adipocyte-to-myofibroblast transdifferentiation.