Systemic sclerosis (SSc) is an autoimmune disease characterized by immune dysfunction, vascular system dysfunction, and tissue fibrosis. Vascular injury, vascular remodeling, and endothelial dysfunction are the hallmark pathological changes of the disease. In the early stages of SSc development, endothelial cell injury and apoptosis can lead to vascular and perivascular inflammation, oxidative stress, and tissue hypoxia, which can cause clinical manifestations in various organs from the skin to the parenchymal organs. Early diagnosis and rational treatment can improve patient survival and quality of life. Ancillary examinations such as nailfold capillaroscopy as well as optical coherence tomography can help early detect vascular injury in SSc patients. Studies targeting the mechanisms of vascular lesions will provide new perspectives for treatment of SSc.