Abstract
Objective:

To describe the efficacy and safety in all patients with systemic sclerosis–associated pulmonary arterial hypertension who started selexipag between 09-2016 and 06-2018 in two pulmonary arterial hypertension expert centers.

Methods:

All patients with systemic sclerosis–associated pulmonary arterial hypertension diagnosed by right heart catheterization and treated with selexipag were included. Every 12 weeks, treatment effect was assessed by (1) the opinion of the expert team and (2) the abbreviated risk assessment, consisting of functional class, six-minute walking distance, and N-terminal prohormone of brain natriuretic peptide level at baseline and during follow-up. Side effects and adverse events were registered.

Results:

We included 13 systemic sclerosis–associated pulmonary arterial hypertension patients, 10 patients were female, median age (interquartile range) of 68 (58–75) years, median systemic sclerosis disease duration of 7.4 (4.7–13.5) years, and median pulmonary arterial hypertension duration of 4 (2.5–7.5) years. Two patients discontinued selexipag within 4 weeks due to side effects. The remaining 11 patients had a median follow-up duration of 48 (interquartile range = 24–72) weeks. Two patients died (one pulmonary arterial hypertension–related, the other systemic sclerosis–related). According to the expert team, 8 of 11, 9 of 10, and 5 of 7 patients stabilized or improved at 12, 24, and 48 weeks, respectively. According to the abbreviated risk assessment at study end, 3 of 11 patients had 1 low-risk criterion. No previously unrecorded side effects were reported.

Conclusion:

Adding selexipag to background therapy in a high-risk cohort of systemic sclerosis–associated pulmonary arterial hypertension patients provided sustained stabilization of symptoms with an acceptable safety profile. Improvement was reached in only two of our patients. Further research should focus on systemic sclerosis–associated pulmonary arterial hypertension patients treated with multiple targeted treatments, preferably these patients should be prospectively followed in international registries.

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