Abstract
Data on macrovascular involvement in systemic sclerosis (SSc) are still debatable. The aim of this study was to estimate its prevalence and possible determinants in a large cohort. One hundred and fifty-five outpatients with SSc were enrolled. Data about disease characteristics and cardiovascular risk factors were collected and patients underwent ecocolor Doppler ultrasonography of arteries of the neck and lower (LL) and upper (UL) limbs. Mean age was 57.9±14.5years and most were female (88.4%) with a limited subset (63.2%). Mean disease duration was 11.4±8.1years. Twenty-three (14.8%) had hypertension, 7 (4.8%) diabetes, 64 (41.3%) hypercholesterolemia and 63 (40.6%) were active/past smokers. Seventy-nine (49%) patients had plaques at carotids, 49 (32.9%) at LL and 7 (4.9%) at UL. In multivariate analysis, patients with carotid plaques had more often a limited pattern (P=.001), patients with distal LL plaques pulmonary arterial hypertension (P=.006) and patients with proximal LL plaques lower diffusing capacity for carbon monoxide adjusted to hemoglobin and its ratio to alveolar volume (P=.004). In patients with UL plaques traditional cardiovascular risk factors were not more common, while forced vital capacity was lower (P=.023). Finally, upper limb and proximal LL plaques were as common in early disease patients as in longstanding ones, although the former were younger. This study shows that macrovascular involvement is quite common in SSc and that some disease characteristics linked to microvascular involvement are associated with atherosclerotic plaques, which can be present even in early disease. Our study suggests that a complete evaluation of macrocirculation is mandatory for rheumatologists treating SSc patients.
Highlights
Skin and visceral microvasculopathy is a typical characteristic of systemic sclerosis (SSc), together with abnormal widespread deposition of collagen and other proteins of extracellular matrix, as shown by some threatening and severe clinical manifestations such as digital ulcers, pulmonary artery hypertension (PAH) and scleroderma renal crisis.[1,2]Whether macrovasculopathy affects scleroderma patients has been the object of some studies leading to contrasting results
In this study we have evaluated the macrovascular involvement in patients with SSc by performing a Doppler ultrasonography (DUS) of carotid, upper limbs (UL) and lower limbs (LL) and by collecting information on disease and cardiovascular risk factors (CRF)
We have found that macrovascular involvement is quite common and that traditional CRF and some disease characteristics are associated with the development of plaques, in the univariate analysis that may be affected by age and disease duration, and in multivariate models
Summary
Skin and visceral microvasculopathy is a typical characteristic of systemic sclerosis (SSc), together with abnormal widespread deposition of collagen and other proteins of extracellular matrix, as shown by some threatening and severe clinical manifestations such as digital ulcers, pulmonary artery hypertension (PAH) and scleroderma renal crisis.[1,2]Whether macrovasculopathy affects scleroderma patients has been the object of some studies leading to contrasting results. A possible reason is the great heterogeneity between studies, as underlined by a meta-analysis published in 2011.3 The same paper showed that carotid intima-media thickness (cIMT) was found higher in SSc than controls in 6 out of 14 studies.[3] In those showing higher cIMT, differences with controls were found comparable to those shown in other diseases characterized by an increased cardiovascular risk such as rheumatoid arthritis (0.09 mm),[4] diabetes mellitus (0.13 mm)[5] and familial hypercholesterolemia (0.12 mm),[6] so the burden of carotid atherosclerosis in SSc may be of some relevance, still debatable. Ho et al[7] found evidence of atherosclerosis in 9 out of 53 SSc cases (17%) compared with no controls, whereas Bartoli et al[8] and Nordin et al[9] did not show any difference in ankle-brachial pressure index (ABPI) between patients and healthy subjects
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