[Systemic sclerosis and macrovascular involvement: Status of the issue in 2019]

Systemic sclerosis (SSc) is a rare immune disease leading to fibrosis of the skin and internal organs. Microvasculopathy is a hallmark of SSc. However, some patients have severe macrovascular complications as affecting cerebral, cardiac or peripheral vessels. To date, macrovascular involvement in SSc remains a matter of debate. Many studies have shown an increased prevalence of macrovascular involvement in SSc in comparison with controlled subjects with similar cardiovascular risk factors. Various methods were used: ankle brachial pressure index, intima media thickness, imagery, coronary calcium score, pulse wave velocity, or flow mediated dilation. The pathophysiology of macrovascular involvement remains unknown and is probably multifactorial: accelerated atherosclerosis, endothelial dysfunction, or reflected wave of microvessel obliteration. The aim of this study was to perform a comprehensible review of the literature, through the study of different types of involved vessels. Results of the main studies are summarized in tables according to the method of investigation used.