Frequency of PAH and its impact on survival in Systemic Sclerosis
The prevalence of RHC-confirmed SSc-PAH is estimated to be 10% to 15%. The presence of PAH in a scleroderma patient has a devastating impact on survival. Prior to the availability of PAH-specific therapies, the 5-year survival was 10% for SSc patients with PAH compared with 80% for SSc patients without PAH. The presence of advanced disease (functional class III-IV) portends a particularly poor outcome. Over the past 15 years, multiple PAH-specific therapies have become available and their implementation has led to clinical improvement and an overall improved prognosis in patients with SSc-PAH compared with historical controls.
