Screening for pulmonary arterial hypertension in systemic sclerosis: Now or never!

Abstract

Systemic sclerosis (SSc), a chronic multisystem autoimmune disease characterized by fibrosis of theskin and internal organs and vasculopathy, has a high burden of mortality. One of the major contributorsto mortality in patients with SSc is pulmonary arterial hypertension (PAH), which affects up to10% of individuals and results in up to 15 years of life loss. Best practice recommendations are forasymptomatic patients with SSc and SSc-spectrum disorder to be screened annually for the earlydetection of SSc-PAH. Recently published data from large registries have shown improvements inthe long-term outcomes in patients who are diagnosed with SSc-PAH because of systematic annualscreening. This review will address the current clinical and research implications of the screening forthe early detection of SSc-PAH.