Fig. 1

Pathophysiology of Systemic Sclerosis (SSc). In the pathophysiology of SSc first of all, an immune dysregulation is caused that is displayed by auto antibody production. The mechanism during which the dyscrasias is caused, is not completely understood, but it is proposed that genetics and the environmental factors play important roles in the process. Autoantibodies as well as other factors like high levels of reactive oxygen species(ROS) cause vascular injury, the process that leads to the activation of fibroblasts. Activated fibroblasts produce an unusual high amount of collagen (especially type I) as well as other components of extracellular matrix (ECM) that form an extensive amount of fibrosis (Velier et al. 2019)