Normal cardiovascular autonomic function in patients with systemic sclerosis (CREST variant)

Sixteen consecutive patients with systemic sclerosis (CREST variant (calcinosis, Raynaud's pnenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia)) were included in this study. Standard non-invasive clinical and electrocardiographic procedures for the detection of cardiovascular autonomic insufficiency were performed in all patients and included heart rate (HR) response to deep breathing, Valsalva manoeuvre, and standing, and blood pressure (BP) response to hand grip and standing. None of the patients had overt cardiovascular disease or were receiving cardiovascular treatment at the time of testing. Results were compared with those obtained in 17 controls. A significant difference between patients and controls was observed only in the HR and BP response to standing (results of this last procedure were within the normal expected values in both patients and controls). We conclude that patients with systemic sclerosis and CREST syndrome may have an abnormal vascular response to standing; cardiovascular autonomic insufficiency, however, does not appear to be a significant feature in these patients.