Practical considerations in the management of inhaled prostacyclin therapy for pulmonary hypertension associated with interstitial lung disease (WHO group 3)

Abstract

Pulmonary hypertension (PH), as a consequence of lung disease or hypoxia, has been classified as Group 3 PH by the World Symposium on Pulmonary Hypertension. The most common lung diseases associated with Group 3 PH are chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD). PH in ILD (PH-ILD) is associated with reduced exercise capacity, greater supplemental oxygen needs, decreased quality of life, and earlier death compared to ILD alone. Several agents have been evaluated in clinical trials for the treatment of Group 3 PH, but only one treatment has been recently approved by the FDA as conclusively demonstrating efficacy for the treatment of pulmonary hypertension in this group. In the INCREASE study, treprostinil inhalation solution (Tyvaso) demonstrated significant clinical benefit for patients with PH-ILD. The inhaled route of administration may be associated with cough, throat irritation, pharyngolaryngeal pain and risk of bronchospasm and are important considerations upon initiation of therapy. Here we provide a practical review of inhaled prostacyclin therapy and suggestions for healthcare professionals to optimize the management and outcomes for the treatment of WHO Group 3, PH-ILD patients. Recommendations include up-to-date practical considerations pertaining to the entire care team and encompass patient education and communication, monitoring, titration methods and mitigation of side effects.

Keywords: Administration; Adverse event management; Inhaled treprostinil; Interstitial lung disease; Pulmonary hypertension; Titration; Tolerability.