Scleroderma Pathogenesis

Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities

-Relationship between endothelial cell (EC) injury and fibrosis in systemic sclerosis (SSc). Injured ECs contribute to the development of fibrosis through several mechanisms, including apoptosis, EndoMT and activation and resultant secretion of pro-fibrotic mediators capable of recruitment and activation of dermal fibroblasts toward the myofibroblast phenotype. EndoMT = endothelial mesenchymal transition. 

-Molecular mechanisms controlling fibroblast activation and regulation of relevant gene expression in SSc patients. Gene expression of collagens and other ECM molecules by fibroblasts is dysregulated in SSc patients, through soluble paracrine/autocrine mediators, ROS, and biomechanical properties of the surrounding ECM. 

journals.sagepub.com

The “myth” of loss of angiogenesis in systemic sclerosis: a pivotal early pathogenetic process or just a late unavoidable event?

Systemic sclerosis is considered a disease…
arthritis-research.biomedcentral.com