The pathophysiology of systemic sclerosis (SSc) is characterized by a cascade of microvascular injury with apoptosis of endothelial cells, Th2/M2 biased inflammation and autoimmunity and aberrant fibroblast activation with excessive… Click to show full abstract
The pathophysiology of systemic sclerosis (SSc) is characterized by a cascade of microvascular injury with apoptosis of endothelial cells, Th2/M2 biased inflammation and autoimmunity and aberrant fibroblast activation with excessive matrix deposition. We increasingly recognize that physiological wound healing and the initial stages of SSc share many commonalities. However, in contrast to physiological repair, these tissue repair programs are not effectively terminated in SSc resulting in progressive matrix deposition and tissue damage. The mechanisms of fibroblast activation may change during the cause of SSc; Profibrotic mediators released from infiltrating leukocytes, activated endothelial cells and degranulated platelets may drive fibroblast activation and collagen release in particular in early stages, whereas endogenous activation loops with activation of fibroblasts by epigenetic changes and biomechanical or physical factors may be of increasing importance in later stages of SSc. In my presentation, I will discuss novel insights into the pathogenesis of SSc with a particular focus on the interplay of inflammation and fibroblast activation. I will focus on translational aspects and highlight mechanisms with high potential for transfer from bench-to-bedside. Disclosure of Interests: None declared
               
Click one of the above tabs to view related content.