The oral drug selexipag is beneficial and safe for patients with pulmonary arterial hypertension (PAH) and connective tissue disease (CTD), according to a study published in Rheumatology and Therapy. 

The most common cause of PAH is idiopathic; the second is a CTD such as systemic sclerosis or systemic lupus erythematosus. The association between systemic sclerosis and PAH, in particular, is well-documented in the medical literature. Patients with both PAH and CTD are more likely to have a poor prognosis. Studies also indicate that patients with both disorders respond less effectively to treatment. 

Selexipag is a selective prostacyclin receptor agonist. A landmark trial, the GRIPHON trial, included 649 patients with idiopathic PAH and 334 patients with CTD-associated PAH and found that oral selexipag reduced the risk of morbidity and mortality by 40% compared with the control. While this study suggests that patients with CTD-associated PAH can benefit from selexipag, there are no studies of its use in real-world clinical practice. The authors of the current study aimed to assess the impact of oral selexipag on PAH clinical outcomes using real-world data. 

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The research team used the Optum de-identified Clinformatics Mart Database, which contains comprehensive data on a large number of patients according to disease category. They analyzed the data of adult patients with PAH who had at least 1 claim for oral selexipag between December 2015 and December 2019. Selected patients were then stratified into 2 cohorts: those with CTD comorbidities and those without. The primary outcomes of the study were time to first all-cause hospitalization and time to first PAH-related hospitalization. 

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Three hundred seventeen patients were identified; 237 had CTD comorbidities and 80 did not. Among patients with CTD-associated PAH, the median time to all-cause and PAH-related hospitalization was 450 and 484 days, respectively. Among patients with idiopathic PAH, the median time to all-cause and PAH-related hospitalization was 493 and 503 days, respectively. In summary, there was no statistically significant difference between the 2 cohorts with regard to the primary outcomes of the study. 

Overall, this study found that the treatment benefits (such as reduction in the risk of disease progression and hospitalization) were consistent for patients with CTD-associated PAH as well as patients with PAH not associated with CTD. 

“The results from this study corroborate the findings of the GRIPHON post hoc analysis of PAH-associated CTD patients and the benefit associated with selexipag use in PAH-CTD patients,” the authors concluded. 


Tsang Y, Singh R, Verma S, Panjabi S. Hospitalization among pulmonary arterial hypertension patients with and without connective tissue disease comorbidities prescribed oral selexipagRheumatol Ther. Published online March 23, 2023. doi:10.1007/s40744-023-00547-z