A “special new” cluster of patients with systemic sclerosis (SSc) has been observed in whom the coexistence of pulmonary arterial hypertension (PAH) and subclinical coronary atherosclerosis (SCA) has been reported, according to findings from a prospective study published in the European Journal of Internal Medicine.

Microvascular abnormalities in all tissues are the hallmarks of SSc, but increased interest in the involvement of cardiovascular (CV) disease has been reported recently. Despite coronary angiography continuing to be the gold standard for evaluating coronary artery disease, new alternative markers for the presence of SCA, including the coronary calcium score, appear to be useful as predictors of increased risk for CV disease events among individuals with SSc.

In the current study, the researchers sought to:

  • Evaluate the frequency of SCA among individuals with SSc via use of computed tomography (CT), as well as assess the differences in clinical, CV, and demographic profiles between those with SCA and those without SCA, as measured by coronary calcium score using CT scans
  • Establish those predictors related to major CV events over 5 years of follow-up in patients with SSc
  • Confirm the performance of CV risk scores in patients with SSc for detection of SCA and major CV events

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Sixty-seven consecutive patients with SSc who were asymptomatic for CV symptoms were enrolled in the study at the Rheumatologic Clinic, University of Foggia in Italy, between January 2017 and December 2017. All patients were followed until January 2023. Study inclusion criteria were as follows:

  • 18 years of age or older
  • Fulfillment of American College of Rheumatology/European Alliance for Associations for Rheumatology classification criteria
  • Disease duration more than 1 year
  • Recent (ie, <6 months) evaluation for interstitial lung disease and PAH

The mean participant age was 59.2±9.1 years;  the median duration of disease was 7 years. Overall, 90% (60 of 67) of the patients were females.

Results of the study showed that the prevalence of SCA in the study participants with SSc was 42%, with Agatston scores of 59.5 units. The following statistically significant findings were reported among individuals with SCA compared with those without SCA:

  • Older age: 66.0±1.8 vs 52.6±5.8 years, respectively; P =.0001
  • Higher rates of centromere protein B antibodies: 57% vs 26%, respectively;
    P =.009
  • Higher rates of PAH: 25% vs 3%, respectively; P =.008
  • Higher rates of dysphagia: 86% vs 61%, respectively; P =.027
  • Higher rates of statin use: 36% vs 8%, respectively; P =.004
  • Less frequent use of the dual endothelin receptor antagonist bosentan: 11% vs 44%, respectively; P =.009
  • Higher rates of carotid plaque: 82% vs 13%, respectively; P =.0001
  • Higher rates of peripheral artery disease: 79% vs 18%, respectively; P =.0001
  • Higher rates of metabolic syndrome: 25% vs 0%, respectively; P =.002

Regarding classical risk factors for CV disease, no significant differences were observed between patients with SSc and SCA vs those with SSc without SCA in smoking status, body mass index, obesity rates, and lipid profiles.

“A careful assessment of CV involvement in SSc, and a more aggressive therapeutic strategy for preventing CAD and treating PAH, should be highly suggested to reduce major CV events in patients with SSc,” the researchers concluded.


Rotondo C, Sciacca S, Rella V, et al. Subclinical coronary atherosclerosis, detected by computer tomography with coronary calcium score, and the occurrence of major cardiovascular events at 5 years of follow-up in a cohort of patients with systemic sclerosis. Eur J Intern Med. Published online June 12, 2023. doi:10.1016/j.ejim.2023.06.003