[Macrovascular complications of systemic sclerosis: Prospective descriptive Doppler ultrasound study in 20 patients]

Purpose: Systemic sclerosis is characterized by cutaneous sclerosis, vascular disease and immunological dysfunction. The prevalence of macrovascular disease remains controversial.

Patients and methods: This was a descriptive prospective single-center study conducted in the vascular medicine department of the University Hospital of Bordeaux from July 1 to September 1, 2015. All inpatients followed for systemic scleroderma were included. Each patient had a Doppler ultrasound of the supra-aortic, upper and lower limb, and digital arteries as well as the aorta. The main objective of the study was to describe the presence and location of arterial lesions in patients with systemic sclerosis.

Results: Of the twenty patients included, there were 13 women and 7 men; mean age was 58±16years. Patients exhibited wall thickening (n=16, 80%), calcified plaques (n=10, 50%), hemodynamically significant stenoses (n=3, 15%) and arterial occlusions (n=12, 60%). Ankle brachial pressure index (ABPI) was 0.98±0.16 on the right and 0.99±0.21 on the left. Two patients had ABPI<0.8. The mean brachial systolic blood pressure was 113±14mmHg. Arterial mapping (860 arterial sites) found wall thickening (n=93 arteries, 10%), calcified plaques (n=47, 5%), hemodynamically significant stenoses (n=7) and occlusions (n=22). Arterial occlusions were located in the ulnar arteries (n=2), the digital arteries (n=18), the posterior tibial artery (n=1) and the dorsalis pedis artery (n=1).

Conclusion: The data of our study correlate with macrovascular disease described in the literature. This finding raises two questions: how does this concept integrate with the severity of Raynaud's phenomenon and the risk of digital ulcers and changes in patients' capillaroscopic landscape during follow-up? What is the cause of these vascular anomalies, some of which are very different from what is observed in atherosclerosis?