Selexipag Improves Outcomes in Patients With CTD-PAH

Although the prognosis for connective tissue disease–associated pulmonary arterial hypertension (CTD-PAH) is poorer than for other PAH etiologies, use of selexipag in these patients resulted in outcomes similar to those seen in patients with idiopathic PAH (IPAH), according to an abstract presented at the annual CHEST meeting.

The researchers used real-world data for patients with PAH taking selexipag from the ongoing US-based SPHERE registry. These patients were 18 years or older and were followed up for 18 months. The registry includes data from initiation of selexipag (baseline) and quarterly to the end of the follow up. The researchers analyzed disease characteristics, treatment patterns, and outcomes of the first 500 patients.

The cohort of 500 patients was composed of 132 patients with CTD-PAH, 246 with IPAH, and 122 other patients. The median time from PAH diagnosis to initiation of selexipag was 3.3 years for patients with CTD-PAH. Patients with CTD-PAH, compared with those with IPHA, were also more likely to:

• Be female (87.9% vs 72.4%)
• Have a shorter 6-minute walk distance (286.2 meters vs 320 meters)
• Have interstitial lung disease (29.5% vs 6.1%)
• Have reflux (46.2% vs 26.8%)
• Have immune system disorders (83.3%) vs 19.1%)

Selexipag treatment duration was similar for patients newly initiating the drug with CTD-PAH (16.4 months) and IPAH (16.9 months). Discontinuation rates were slightly higher among patients with CTD-PAH (46.3% vs 44.9%). The causes of discontinuation were:

1. PAH worsening–related adverse events (AEs): 19.5% for CTD-PAH and 14.5% for IPAH
2. Selexipag-related AEs: 17.1% in CTD-PAH and 20.3% in IPAH

Compared with baseline, the majority of patients had improved or stable risk with selexipag. At 18 months, 19.7% of patients with CTD-PAH and 12.2% of patients with IPAH had improved risk; 26.5% of patients with CTD-PAH and 41.5% of patients with IPAH had stable risk; and 17.4% of patients with CTD-PAH and 12.6% of patients with IPAH had worsened risk. The median 18-month survival rate was slightly lower among patients with CTD-PAH (71.0%; 95% CI, 53.7%-82.8%) than IPAH (84.4%; 95% CI, 71.6%-91.8%).

The authors concluded that the real-world registry data showed that disease and treatment characteristics were consistent with previous data. “Outcomes for CTD-PAH [patients] receiving selexipag in the real-world setting were broadly similar to those of IPAH,” they wrote.

Reference

Chin K, Chakinala M, Hemnes A, et al. Real-world data for selexipag in patients with connective tissue disease-associated pulmonary arterial hypertension: a SPHERE (selexipag: the users drug registry) analysis. Presented at: CHEST Annual Meeting 2020; October 18-21, 2020. https://journal.chestnet.org/article/S0012-3692(20)34061-7/fulltext