Cardiovascular and venous thromboembolic events in systemic sclerosis: epidemiological analysis of the Clinical Practice Research Datalink

Objectives
Cardiovascular disease is a leading cause of mortality in systemic sclerosis (SSc). We investigated the association between SSc and the occurrence of both cardiovascular and thromboembolic events using the Clinical Practice Research Datalink (CPRD).

Methods
A validated case-ascertainment strategy identified SSc patients in the CPRD. A cohort study design examined rates of coronary arterial disease (CAD), cerebrovascular disease, peripheral artery disease (PAD) and venous thromboembolism (VTE) in SSc patients matched to non-SSc comparators by age, sex and location in a 1:6 ratio. Prevalent and incident cases of SSc were analysed separately. Cox regression analysis was used to determine hazard ratios for event occurrence, adjusted for traditional cardiovascular risk factors.

Results
We identified 877 eligible incident cases of SSc who were matched to 5262 patients without SSc (83.7% female), with a mean follow-up of 8 years. We identified a higher background prevalence of CAD, PAD and VTE at baseline (pre-dating diagnosis) in the SSc cohort. There was a significantly increased risk of CAD, PAD, peripheral venous thromboses and pulmonary embolism during follow-up in SSc compared with the non-SSc group (adjusted hazard ratios between 1.84–3.01), particularly amongst males. There was no increased risk of cerebrovascular disease.

Conclusions
We have identified an increased risk of cardiovascular events and VTE, both prior to a diagnosis of SSc and within 8 years of SSc diagnosis. Work is needed to establish the mechanism of arterial/venous thrombotic events in SSc. This insight will facilitate more targeted and effective preventative strategies and improve outcomes in SSc.