Background/Purpose: Cardiac involvement, due to impairment of coronary microcirculation and myocardial fibrosis, affects prognosis in individuals with SSc, and represents one of the leading causes of death in this population. There is limited data on the risk of atherosclerotic cardiovascular disease (CVD) in SSc. We aimed to compare the prevalence of traditional CVD risk factors and incidence of atherosclerotic CVD events among incident cases of SSc vs age- and sex-matched comparators.

Methods: Medical records of patients with a diagnosis or suspicion of SSc in a geographically well-defined area from Jan 1, 1980 to Dec 31, 2016 were reviewed to identify incident cases of physician diagnosed SSc. Fulfillment of the 1980 and 2013 SSc classification criteria was ascertained. A 2:1 cohort of age- and sex-matched non-SSc subjects from the same population base was randomly selected for comparison. Data on SSc characteristics, traditional CVD risk factors (i.e., smoking status, obesity, hypertension, dyslipidemia, diabetes mellitus [DM]), and CVD events (i.e., coronary artery disease [CAD], peripheral artery disease [PAD], abdominal aortic aneurysm [AAA], cerebrovascular disease [stroke/TIA], and heart failure) were collected. Cumulative incidence was adjusted for the competing risk of death.

Results: The cohort included 79 incident SSc cases and 158 non-SSc comparators (mean age of 56 ± 16 years at diagnosis/index, 90% female for both cohorts; 87% [SSc] and 93% [non-SSc] Caucasian). Mean body mass index was significantly lower in SSc (26.5 ± 5.9 kg/m²) than non-SSc (29.3 ± 8.1 kg/m²; p=0.01). Diabetes mellitus was also less common in SSc than non-SSc at diagnosis/index (3% vs 9%, p=0.05). There were no differences in smoking, hypertension or hyperlipidemia. Prior to SSc diagnosis/index, there was no difference in the prevalence of any CVD events (15 SSc vs 19 non-SSc; p=0.17). During the median follow up of 9.8 y (SSc) and 8.9 y (non-SSc), 19 SSc and 15 non-SSc patients developed CVD events, for a 10-year cumulative incidence of 22.4% among SSc and 10.6% among non-SSc. This corresponded to a 3 fold increased risk (hazard ratio [HR]: 3.25; 95% confidence interval [CI]: 1.64-6.46, p=0.001) in SSc patients vs. comparators. This increased risk was predominately due to CAD (HR: 3.20; 95% CI: 1.56-6.72, p=0.002), but PAD/AAA risk also approached significance (HR: 4.20; 95% CI: 0.99-17.91, P=0.052). There was no evidence of increased risk of cerebrovascular events (HR: 1.06; 95% CI: 0.23-4.94, p=0.94).

Conclusion: Patients with incident SSc have a lower BMI and prevalence of DM than non-SSc comparators. Despite having no significant difference in the prevalence of traditional CVD risk factors and CVD events at baseline, patients with SSc have a >3 fold increase of CVD events after SSc diagnosis when compared to non-SSc comparators, predominately due to CAD. This increased rate warrants a high vigilance for CAD in patients with SSc. It may potentially be related to the increased risk of endothelial dysfunction, microvascular injury and chronic inflammation characteristic of the disease, but warrants further detailed study.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cardiovascular-cv-risk-factors-and-atherosclerotic-cv-events-among-incident-cases-of-systemic-sclerosis-results-from-a-population-based-cohort-1980-2016/