Efficacy and Safety of Selexipag Treatment in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension with Concomitant Interstitial Lung Disease

TL;DR

This study shows that selexipag may improve exercise capacity and reduce lung pressure in patients with connective tissue disease-related pulmonary hypertension and interstitial lung disease.

Contribution

The study is among the first to evaluate selexipag in CTD-PAH patients with concomitant ILD, suggesting its potential efficacy in this challenging group.

Findings

Selexipag increased mean six-minute walk distance by 101.75 m after 16 weeks.

Selexipag significantly reduced estimated right ventricular systolic pressure.

Selexipag decreased supplemental oxygen requirements and stabilized pulmonary function tests.

Abstract

Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and concomitant interstitial lung disease (ILD) are particularly challenging to manage due to concerns about ventilation–perfusion mismatch with systemic vasodilators. In this case series, we evaluated the effects of selexipag in eight prostacyclin-naïve CTD-PAH patients with concomitant ILD. Clinical, functional, and laboratory data were collected at baseline and after 16 weeks of treatment. After 16 weeks of treatment, the mean six-minute walk distance increased by 101.75 m (p < 0.05), and the mean estimated right ventricular systolic pressure decreased significantly (p < 0.05). Mean N-terminal pro b-type natriuretic peptide levels declined by 63%, though this reduction did not reach statistical significance. Importantly, supplemental oxygen requirements trended downward (p < 0.05) and pulmonary function tests remained stable. Pulmonary vasodilators have long been unsuccessfully studied in PH-ILD patients until the INCREASE trial. While other systemic agents used in PAH have not shown as much success as inhaled treprostinil in treating PH-ILD, our case series highlights the potential role of selexipag in patients with concomitant CTD-PAH and ILD. Further investigation of selexipag in pure Group 3 PH-ILD patients is warranted.

Figures