Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) have a higher mortality rate than non-SSc-APAH patients with idiopathic disease as well as those with other connective tissue diseases (CTD-APAH). In a recent study, researchers pinpoint specific predictors that put patients with SSc-APAH at higher risk of death.
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Researcher Lorinda Chung, MD, MS, a rheumatologist and allergist at Stanford University in Stanford, Calif., and colleagues, used the Registry to Evaluate Early and Long-Term PAH Management (REVEAL) patient population to identify mortality predictors among patients with SSc-APAH and non-SSc-CTD-APAH patients with previously or newly diagnosed PAH. The results of their analysis were published online in the July 3, 2014, issue of Chest.
Chung et al call their paper the first to identify low baseline systolic blood pressure (SBP) ≤ 110 mmHg as an independent predictor of death in patients with SSc-APAH. Additional risk factors for these patients include being a male older than 60 years; having poor exercise capacity, measured at a slow, six-minute walking distance (6MWD) <165 meters; and severe hemodynamic indices, including elevated mean right atrial pressure (mRAP) of >20 mmHg and elevated pulmonary vascular resistance >32 Wood units. The investigators note that predictors of mortality for patients with SSc-APAH were similar to core predictors for PAH as a whole.
“Our study provides further evidence that patients with SSc-APAH experience higher mortality rates than patients with other CTD-APAH in both incident and prevalent populations,” the authors write. “Our results validate the utility of the risk score calculator in patients with CTD-APAH, including patients with SSc-APAH.”