In systemic sclerosis (SSc), the involvement of the vascular system dictates the fate of the patient. In recent decades, significant attention has been devoted to understanding the mechanisms involved in the derangement of the endothelium of the microvascular system in SSc. In this perspective, many papers have investigated the consistency of endothelial markers to disclose the ongoing endothelial derangement during the progression of the disease. To date, no endothelial marker has been shown to be useful in a real-world setting, and the search for new methodologies to shed light on this important clinical need is still ongoing.
The endothelial layer is the interface between the bloodstream and the vessel wall. It is well known that any modification of the endothelial function is of pivotal importance in SSc pathogenesis. In fact, endothelial cells (EC) react to any humoral, neural, hemodynamic, and oxidative stress. Moreover, the endothelium affects platelet function, vessel wall inflammation, proliferation and migration of smooth muscle cells, and vascular tone control. Therefore, endothelial derangement is of paramount importance in SSc pathogenesis.