PULMONARY VASCULAR DISEASE

Pulmonary arterial hypertension (PAH) is a pro-gressively fatal disorder that affects patients inde-pendently or in association with other conditions, including systemic sclerosis (SSc). In prospective cohort studies of patients with SSc who develop dyspnea, the prevalence of PAH is between 7.85 % and 12%. 1-3 More than 50 % of deaths in SSc are related to intrin-sic disease manifestations, with the most common being pulmonary fi brosis/interstitial lung disease (ILD) (35%), PAH (26%), and heart failure and arrhyth-mias (26%). 4 In a report from the French ItinérAIR-Sclérodermie study, the 3-year survival of 546 patients Background: The impact of modern therapy on survival in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) is not clear. We sought to determine associations among commonly used clinical and hemodynamic variables, treatment, and long-term survival in PAH associated with SSc compared with PAH defined as idiopathic, familial, or associated wit