Peripheral Macrovascular Involvement in Systemic Sclerosis: A Cohort Study by Color and Spectral Doppler Ultrasonography

Abstract

Objectives: Systemic sclerosis (SSc) is a disease characterized by diffuse sclerosis of skin and organs and small vessel vasculopathy. Despite it, large vessels can also be involved with ulnar artery vasculopathy, revealing as a more frequent feature of SSc. The aim of this paper is to assess the macrovascular involvement of SSc patients through an ultrasound (US) evaluation of radial and ulnar arteries. Methods: Radial and ulnar resistance indices (RIs) and peak systolic velocity (PV) (cm/s) together with clinical features of SSc patients were evaluated. Raynaud phenomenon (RP) and healthy control (HC) groups were used for comparison. Results: Forty-three SSc patients were evaluated. Twelve patients (28%) had ulnar artery occlusions (UAOs). In nine cases (75%), UAOs were bilateral. A high UAO prevalence (42%) was found in SSc patients with late nailfold-video-capillaroscopy (NVC) pattern (p = 0.0264). Patients with UAOs had digital ulcers (DUs) in 10 cases (83.3%). Radial and ulnar PVs were lower in SSc and RP patients than the HC group. Radial and ulnar RIs were higher in SSc and RP patients than the HC group. A decision tree analysis led to the classification of 70% of SSc patients with an ulnar RI > 0.82 and ulnar PV > 2.8 cm/s. The most influential variables on UAO development were interstitial lung disease (ILD) (p = 0.002) and NVC pattern (p = 0.002). A positive correlation was shown between modified Rodnan skin score (mRSS) and ILD (p = 0.283; r = 0.033), mRSS and DU (r = 0.344; p = 0.012) and DU and ILD (r = 0.303; p = 0.024). Male sex was associated with increased UAO frequency (p = 0.042). Conclusions: UAO is a peculiar feature of severe SSc present in 28% of the cases, particularly associated with the presence of ILD and late NVC pattern. In 75% of the cases, UAOs are bilateral. DUs are very frequent in patients with UAOs (83%). The RI evaluated by US could be useful to distinguish SSc from HC patients. US could be a useful tool for assessing high-risk DU development in patients.

Biomarkers in the Pathogenesis, Diagnosis, and Treatment of Systemic Sclerosis

“…New methods to quantify RP beyond nailfold capillaroscopy include laser Doppler imaging, laser speckle contrast analysis, thermal imaging, and color and spectral Doppler imaging. 182 , 183 Pharmacologic therapy for RD has recently been reviewed and is generally initiated first with calcium channel blockers, and if ineffective followed by PDE-5 inhibitors or fluoxetine with endothelin-1 receptor blockers and intravenous prostanoids reserved for the most resistant cases. 133–136 , 182 , 184 …”

“…New methods to quantify RP beyond nailfold capillaroscopy include laser Doppler imaging, laser speckle contrast analysis, thermal imaging, and color and spectral Doppler imaging. 182,183 Pharmacologic therapy for RD has recently been reviewed and is generally initiated first with calcium channel blockers, and if ineffective followed by PDE-5 inhibitors or fluoxetine with endothelin-1 receptor blockers and intravenous prostanoids reserved for the most resistant cases. [133][134][135][136]182,184 Vascular injury and subsequent fibrosis may occur from the activation and apoptosis of endothelial cells, specific autoantibodies, infectious agents, endogenous hypercoagulability, reactive oxygen species, as well as other causes providing many potential biomarkers (Table 4).…”