Association of Systemic Sclerosis With Incident Clinically Evident Heart Failure

Primary myocardial involvement is an important cause of death in systemic sclerosis (SSc). Subclinical diastolic/systolic heart dysfunction is recognized; however, whether this indicates a subsequent increased risk of clinically overt heart failure (HF) remains largely unknown. We aimed to investigate the risk of clinically overt HF in a large, unselected SSc cohort.

This matched, retrospective cohort study was conducted using a nationwide insurance database in Taiwan. Incident SSc patients with no history of HF were identified, and non-SSc comparison groups were selected and matched to the SSc groups by age, sex, and cohort entry time. The cumulative HF incidence was estimated using the Kaplan-Meier method. Multivariable Cox proportional hazards regression was used to calculate adjusted hazard ratios (HRs) for HF hospitalization.

A total of 1,830 SSc patients and 27,981 controls were identified. The cumulative incidence of hospitalized HF at 3, 5, and 10 years among patients with SSc were 3.5%, 5.3%, and 9.7%, respectively. Compared with non-SSc individuals, SSc patients had an increased risk of HF (adjusted HR 3.26 [95% confidence interval (95% CI) 2.49-4.28]). Subgroup analyses revealed that the impact of SSc on the occurrence of HF was greater among patients ages <50 years than those ages ≥50 years (HR 7.8 [95% CI 4.03-15.1] versus HR 2.78 [95% CI 2.06-3.76]).

SSc is associated with a markedly higher risk of clinically evident HF and not asymptomatic ventricular dysfunction alone. These findings provide real-world evidence suggesting the use of appropriate screening strategies to detect these lethal complications early in SSc.