BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH.METHODS: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of SSc patients at high risk for or with incident pulmonary hypertension based on right heart catheterization (RHC). Incident World Health Organization Group I PAH patients were analyzed. Kaplan-Meier survival curves were generated for the overall cohort and those who died of PAH. Multivariate cox regression models identified predictors of mortality.RESULTS: Survival in 160 incident SSc-PAH patients at 1, 3, 5, and 8-years was 95%, 75%, 63%, and 49%, respectively. PAH accounted for 52% of all deaths. When restricted to deaths due to PAH, respective survival rates were 97%, 83%, 76%, and 76%, with 93% of PAH-related deaths occurring within 4 years of diagnosis. Male sex (HR 3.11 95%CI 1.38-6.98), diffuse disease (HR 2.12 95%CI 1.13-3.93), systolic pulmonary artery pressure (PAP) on echocardiogram (HR 1.06 95%CI 1.01-1.11), mean PAP on RHC (HR 1.03 95%CI 1001-1.07), six-minute walk distance (HR 0.92 95%CI 0.86-0.99), and diffusing capacity for carbon monoxide (HR 0.65 95% CI 0.46-0.92) significantly impacted survival on multivariate analysis.CONCLUSION: Overall survival in PHAROS was higher than other SSc-PAH cohorts. PAH accounted for more than half of deaths and primarily within the first few years after PAH diagnosis. Optimization of treatment for those at greatest risk of early PAH-related death is crucial.
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