In 40 patients (36 females, mean age 56±13 years) with capillaroscopic pattern of sclerodermia (SSc) and 30 matched healthy subjects (27 females), we assessed endothelium-dependent (flow-mediated dilation, FMD), and -independent vasodilation (sublingual administration of glycerol trinitrate, GTN, 25 μg), by ultrasounds and computerized analysis. Augmentation index (AIx) and brachial pulse wave velocity (PWV) were assessed by applanation tonometry (Sphygmocor^®). Disease activity and severity scores and inflammatory parameters (erythrocyte sedimentation rate, ESR; C reactive protein, CRP; interleukin-6, IL-6) were evaluated.

FMD was significantly (p < 0.001) lower in SSc (4.3±2.3%) than in controls (6.9±3.3%), while response to GTN was similar. FMD was not related to disease’s activity and severity score or CRP and IL-6 levels. AIx (26.0±13.1 vs 24.8±7.5% %) and PVW (8.8±1.4 vs 8.3±1.4m/s) were not significantly different in SSc as compared to controls. However, AIx was significantly (p < 0.05 or less) related to activity (r = 0.30) and severity score (r = 0.30), ESR (r = 0.32), but not with CRP and IL-6 levels. AIx was related to age (r = 0.65), total cholesterol (r = 0.49) and systolic blood pressure (PAS, r = 0.33). PWV was significantly (p < 0.05 or less) related to severity score (r = 0.49), CRP (r = 0.47) and total cholesterol (r = 0.49) levels, but not to activity score, ESR or IL-6 levels.

In conclusion, SSc patients show marked endothelial dysfunction in the peripheral macrocirculation, which is not related with disease’s grade. Upper limb arterial stiffness seems not to be affected by SSc, but increased indexes of arterial stiffening are related to greater disease activity and severity, inflammatory markers and cardiovascular risk factors.