Objectives: Systemic sclerosis (SSc) is an autoimmune inflammatory, fibrotic disorder in which vascular dysfunction is a major manifestation. Other auto-immune diseases, like rheumatoid arthritis and systemic lupus erythematosus, are complicated by excess cardiovascular morbidity and mortality which cannot be explained by traditional risk factors alone. SSc is also characterized by increased mortality rates in which cardiovascular deaths play an important role. Previous studies have shown an increased prevalence of macrovascular disease in patients with SSc, but did not correct for traditional risk factors. The aim of our study was to evaluate whether patients with SSc have an increased prevalence of atherosclerosis and to evaluate if disease related factors besides traditional risk factors are implicated in atherosclerosis.
Methods: 50 SSc patients (8 men; mean (± SD) age 55.6 (± 11.6) years) with diffuse cutaneous SSc (4), limited cutaneous SSc (45) en localized SSc (1) and 32 healthy controls (3 men; mean (± SD) age 50.9 (± 10.1) (p=0.054) years were studied. Common carotid intima-media thickness (IMT) was measured by ultrasound. Traditional risk factors were determined in all individuals. Serum levels of vascular cell adhesion molecule-1 and thrombomodulin, and plasma levels of von Willebrand factor were determined as marker of endothelial cell dysfunction.
Results: Mean IMT was increased in SSc patients (mean (± SD) 0.76 (± 0.76) mm) compared to controls (mean (± SD) 0.66 (± 0.11) mm) (p=0.032). A trend was noted towards a higher age (p=0.054) and higher frequency of treated hypertension (p=0.06) in SSc patients, but otherwise no statistic differences in traditional risk factors were found. Controls showed a significantly higher level of the endothelial activation marker VCAM-1, but no differences were seen in the other endothelial markers. Univariate analysis of risk factors showed a correlation of IMT and age (r=0.28, p=0.058 in SSc patients and r=0.409, p=0.025 in controls). No other correlations were found. In a subanalysis of SSc patients with or without increased IMT an increased prevalence of a positive family history for cardiovascular disease was found in the subgroup with increased IMT (p=0.008). In the subanalysis no other differences in traditional risk factors, in disease characteristics and markers for endothelial dysfunction were found.

Conclusion: This study showed an increased IMT in SSc patients compared to healthy controls. Though no differences were seen in traditional risk factors, a subanalysis showed an increased prevalence of a positive family history of cardiovascular disease in the SSc patients with increased IMT. Therefore, besides the vasculopathy, traditional risk factors appear to play a role in the prevalence of atherosclerosis in SSc patients.