Role of vasculopathy in the pathogenesis of systemic sclerosis

Abstract

Systemic sclerosis (SSc) is an autoimmune disorder characterized by skin sclerosis and internal organ fibrosis. Although its etiology and pathogenesis are unclear, the main pathological features of SSc are vasculopathy, aberrant immune activation and tissue fibrosis. Increasing studies have indicated that vasculopathy may be an initial event in the occurrence of SSc, including small vessel spasm, intimal thickening, reduction of vessel number and so on. This review summarizes recent research progress in SSc-related vasculopathy, including the disarrangement of pro-angiogenic factors (e.g. vascular endothelial growth factor and stromal cell-derived factor-1) , angiostatic factors (e.g. endostatin and pentraxin 3) and their receptors (e.g. vascular endothelial growth factor receptor and urokinase-type plasminogen activator receptor) , aberrant number and function of vascular endothelial progenitor cells and vascular endothelial cells, imbalance in the regulation of vascular tone, and dysfunction of platelets and their releasers in SSc. Key words: Scleroderma, systemic; Peripheral vascular diseases; Endothelial cells; Stem cells; Blood platelets