Inhaled Treprostinil for PH-ILD: Comprehensive Review Synthesizes Advances, Challenges, and Future Directions

Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a severe complication significantly increasing morbidity and mortality. Current treatments often fall short, necessitating therapies that address both pulmonary vascular remodeling and potential underlying fibrosis. Inhaled treprostinil (iTre) has emerged as a promising option, offering pulmonary vasodilatory, antiproliferative, and potentially anti-fibrotic effects, while minimizing systemic side effects due to its localized delivery. This review synthesizes the evolving understanding of iTre's role in this complex condition.

This comprehensive review synthesizes the current scientific literature on inhaled treprostinil for pulmonary hypertension associated with interstitial lung disease (PH-ILD). The authors systematically discuss the therapeutic advances, persistent challenges in its clinical application, and critical future research directions. The review aims to provide a holistic perspective on iTre's mechanism, efficacy, and safety profile, drawing insights from existing clinical trials and real-world data to inform future management strategies.

The review highlights inhaled treprostinil as a key therapeutic option for PH-ILD, leveraging its established pulmonary vasodilatory and antiproliferative properties. It also explores the emerging evidence for iTre's potential anti-fibrotic effects, which could address a critical aspect of ILD pathology. The authors discuss the complexities of integrating iTre into existing treatment paradigms, considering factors like patient adherence, device limitations, and the management of potential side effects, which are crucial for real-world outcomes. Future directions emphasize the need for optimized dosing strategies, identification of ideal patient populations, and further elucidation of iTre's long-term impact on disease progression and survival in PH-ILD. > The synthesis emphasizes the need for further research to optimize dosing strategies, identify ideal patient populations, and fully elucidate iTre's long-term impact on disease progression and survival in PH-ILD.