Selexipag Associated With Reduced Morbidity, Mortality Risk in Connective Tissue Disease-Associated PAH

Pulmonary Hypertension Diseased Artery
Pulmonary Hypertension Diseased Artery
In patients with connective tissue disease-associated pulmonary arterial hypertension, selexipag may reduce risk for morbidity and mortality.

Selexipag is well tolerated and reduces morbidity and mortality risk in patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD), according to an exploratory analysis published in The European Respiratory Journal.1

The primary composite end point was the time from randomization to first morbidity or mortality event. The study included 334 patients from the double-blind randomized phase 3 GRIPHON trial (Selexipag [ACT-293987] in Pulmonary Arterial Hypertension; identifier: NCT01106014). GRIPHON included 82 patients with PAH-SLE, 37 with PAH-mixed CTD, and 45 with unspecified CTD. Researchers randomly assigned patients 1:1 to selexipag (n=167) or placebo (n=167).

Patients were included in the following dose groups based on their individualized maintenance dose: low (200 and 400 μg twice a day), medium (600, 800, and 1000 μg twice a day), and high (1200, 1400, and 1600 μg twice a day).

Compared with placebo, patients who received selexipag experienced a 41% reduced risk for the primary composite end point (hazard ratio [HR], 0.59; 95% CI, 0.41-0.85). In patients with PAH with systemic sclerosis and PAH with systemic lupus erythematosus, there was a 44% and 34% risk reduction associated with selexipag compared with placebo, respectively (HR, 0.56; 95% CI, 0.34-0.91 and HR, 0.66; 95% CI, 0.30-1.48).

Disease progression, PAH, and right heart failure represented the most frequent causes of death in this patient population. There was a consistent treatment effect regardless of CTD subtype (P =.89) or baseline PAH therapy (P =.87).

The researchers noted that this analysis was prone to a few limitations. For example, the analyses in this research were exploratory and there was no adjudication for classification of CTD subtype.

Multiple PAH therapies may be an effective treatment strategy in patients with PAH-CTD, the researchers concluded. 

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Gaine S, Chin K, Coghlan G, et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1602493.