Newly Diagnosed PAH Patients Benefit from Selexipag Treatment

Vallerie McLaughlin, MD

New data presented at the CHEST 2021 Annual Meeting suggested that appropriate use and timing of the selective oral IP prostacyclin receptor agonist selexipag could result in the optimization of pulmonary arterial hypertension (PAH), especially for patients who are not at treatment goal.

The data were presented by leading study author Vallerie McLaughlin, MD, director of the Pulmonary Hypertension Program, University of Michigan.

Selexipag had previously been approved for the treatment of PAH to delay disease progression and reduce the risk of hospitalization.

The agonist also achieved a greater reduction in the risk of a morbidity/mortality event compared to placebo in phase 3 of the GRIPHON trial.

In the present analysis, McLaughlin described data from SPHERE, an ongoing, US, multicenter, prospective registry of patients treated with selexipag. The registry collected data on demographic and disease characteristics, as well as outcomes and dosing regimens.

The team evaluated the clinical characteristics and treatment patterns in patients with World Health Organization (WHO) Group 1 PAH initiating selexipag from the time of initiation since diagnosis.

The Methods

A total of 829 patients were enrolled in the study, 652 of whom were previously diagnosed with PAH and 107 who were newly diagnosed.

Of the total number of patients, 759 were diagnosed with WHO group 1 PAH patients, the majority of whom initiated selexipag more than 24 months (58.8%) after initial diagnosis. All but 2 of those patients had at least 1 follow-up assessment.

Data were collected regarding selexipag initiation and quarterly at routine clinic visits for up to 18 months.

Clinical characteristics were analyzed in patients grouped by time on initiation since their diagnosis and change in risk post-initiation was summarized by time since diagnosis.

One-year mortality risk was based on the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk calculator, and risk scores were calculated for every patients irrespective of missing variables.

The Findings

Investigators reported that the median duration of selexipag treatment was 18.5 months, and disease characteristics indicated that PAH was severe in the overall population.

Risk was higher in newly diagnosed patients at the initiation of selexipag; investigators noted that risk was low in 23%, intermediate in 32%, and high in 45% of newly diagnosed patients.

In previously diagnosed patients, risk was low in 46%, intermediate in 30%, and high in 25%.

Additionally, during the follow up, risk scores improved in 32% and 18% of newly and previously diagnosed patients, respectively.

McLaughlin and colleagues recorded some differences in the use of non-PAH-specific medication at baseline, such as fewer patients in the newly diagnosed subgroups were prescribed a selective serotonin reuptake inhibitor (7.5% versus 15.2%, respectively).

Overall, newly diagnosed patients had PAH that was more severe than expected to be treated with an oral therapy, more severe disease and higher risk status, had received less combination therapy, and had a higher proportion of patients with improvement in risk status following selexipag initiation.

“These findings demonstrate that there are opportunities to improve PAH treatment in real-world clinical practice, especially for patients who are not at target risk status,” the team wrote.