Selexipag improves outcomes in PAH-associated connective tissue disease in real-world study
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SAN FRANCISCO — New real-world data demonstrate treatment with selexipag improved outcomes in patients with pulmonary arterial hypertension associated with connective tissue disease.
At the American Thoracic Society International Conference, Sean Gaine, MD, with the National Pulmonary Hypertension Unit at Mater Misericordiae Hospital in Dublin, Ireland, and colleagues reported patient characteristics, treatment patterns and outcomes of patients with PAH-associated connective tissue disease (PAH-CTD) initiating selexipag (Uptravi, Actelion) in real-world settings. The analysis included patients from the EXPOSURE study who initiated selexipag and who had follow-up information through the data cut-off in November 2021.
“These real-world data complement the data from the GRIPHON post hoc analysis, showing improved outcomes in PAH-CTD patients treated with selexipag, including as part of a triple therapy regimen, versus placebo,” Gaine and colleagues wrote in the poster.
The analysis included 133 patients with PAH-CTD. Compared with patients with idiopathic PAH (n = 280) as a reference, those with PAH-CTD were more likely to be women (89% vs. 66%), older (median age, 67 years vs. 61 years) and have more severe functional impairment (WHO functional class III/IV, 67% vs. 66%) at the time of selexipag initiation, according to the results. A higher proportion of patients with PAH-CTD had anemia compared with those with idiopathic PAH.
Three-quarters of patients with PAH-CTD had systemic sclerosis, 8% mixed CTD, 7% systemic lupus erythematosus, 5% rheumatoid arthritis, 3% undifferentiated CTD and 3% primary Sjögren’s syndrome.
Selexipag initiation occurred primary as part of a triple combination therapy in both patients with PAH-CTD and idiopathic PAH.
The incidence of all-cause hospitalizations (44.5 per 100 person-years vs. 26.7 per 100 person-years) and the rate of all-cause mortality (18% vs. 16%) were higher in patients with PAH-CTD compared with idiopathic PAH, while PAH-related hospitalizations (20.2 per 100 person-years vs. 17.1 per 100 person-years) occurred at a similar rate, according to the results.
Selexipag was well tolerated by most patients with PAH-CTD, according to the researchers. The safety profile of selexipag in patients with PAH-CTD was similar to the safety profile in patients with idiopathic PAH, they reported. Overall, 42% of patients with PAH-CTD and 30% of patients with idiopathic PAH discontinued selexipag treatment. Between both patient groups, the primary reasons for discontinuation were tolerability/adverse events (PAH-CTD, 46%; idiopathic PAH, 39%), PAH disease progression (PAH-CTD, 13%; idiopathic PAH, 27%) or death (PAH-CTD, 32%; idiopathic PAH, 19%). Regarding adverse events, more patients with idiopathic PAH experienced adverse events compared with patients with PAH-CTD (33% vs. 30%). The most frequent adverse events between both patients with idiopathic PAH and those with PAH-CTD were diarrhea (8% and 7%, respectively), headache (6% and 5%, respectively) and nausea (3% for both).