Purpose of review: During the last few decades, the death rate due to cardiovascular disease in systemic sclerosis (SSc) patients has substantially increased. Whether this is because of accelerated atherosclerosis, similar to what has been demonstrated in rheumatoid arthritis and systemic lupus erythematosus, remains to be established. In this review, the main recent evidence about potential pathogenic mechanisms of accelerated atherosclerosis, microvascular disease and macrovascular disease in SSc, and the results from the surrogate measures for detecting and quantifying subclinical atherosclerosis in SSc patients are reviewed. Recent findings: Significantly higher risk of coronary heart disease in SSc patients compared with the general population has been found in the largest cohort studies. However, no difference in either atherosclerotic plaque occurrence or intima-media thickness was detected. The diagnostic utility of other techniques, such as cardiac MRI, in order to improve detection and characterization of SSc heart disease remains to be determined. Summary: Although microvascular disease is a hallmark of SSc, mechanistic insights explaining the presence of accelerated atherosclerosis, and the presence and extent of macrovascular disease in SSc patients are lacking. Future challenges will be to unravel the genetic, environmental and ethnic determinants of such processes.
- Cardiovascular disease
- Systemic sclerosis