VASCULOPATHY IN SYSTEMIC SCLEROSIS

Systemic sclerosis (SSc) is a complex, heterogeneous, multi-organ disease, the etiology of which is not yet fully understood. Although fibrosis of the skin and major organs is a well-known, characteristic sign of this condition, the primary dysfunctions contributing to the disease phenotype appear to be vascular and immune in nature. The mechanisms leading to vasculopathy in SSc include dysregulation of vascular tone and endothelial dysfunction, followed by vascular remodeling, as well as abnormal angiogenesis and vasculogenesis. In addition, coagulopathy also plays a role in the disease process. Together, these factors produce a form of vasculopathy specific to SSc, characterized by vascular intimal proliferation in the macro- and micro-circulation, as well as qualitative and quantitative capillary changes.