ATS 2025: Antifibrotic Therapy Combined with Pulmonary Vasodilator Therapy May Improve Survival in Patients with Idiopathic Pulmonary Fibrosis or Other Fibrotic ILD and Pulmonary Hypertension

Introduction:

Pulmonary fibrosis is a progressive interstitial lung disease linked to poor prognosis. Severe fibrotic disease may lead to pulmonary hypertension(PH), worsening morbidity and mortality. Although antifibrotics and pulmonary vasodilator therapies are individually effective for pulmonary fibrosis and pulmonary hypertension, respectively, the benefit of combining these therapies in patients with both diseases remains unclear.

Methods:

A retrospective cohort study was performed involving patients with pulmonary fibrosis—including idiopathic pulmonary fibrosis and other fibrotic interstitial lung diseases (ILDs)—with pulmonary hypertension confirmed by right heart catheterization. Participants received antifibrotic treatment either alone or in combination with pulmonary vasodilators. Transplant-free survival was assessed and compared between patients treated with antifibrotics alone (n=90) and patients receiving both antifibrotic and pulmonary vasodilator therapies (n=16).

Results:

Kaplan-Meier analysis demonstrated that patients receiving a combination of antifibrotic, and pulmonary vasodilator therapy had better transplant-free survival compared to those on antifibrotic therapy alone (25% vs. 0%, log-rank p < 0.001). Treatment with antifibrotic and pulmonary vasodilator therapy was  significantly and independently linked to a lower risk of death or lung transplantation. (HR 0.36, 95% CI 0.19–0.64, p < 0.001).

Conclusion:

A potential survival benefit was observed when pulmonary vasodilator therapy was added to antifibrotic therapy in patients with pulmonary fibrosis or other fibrotic interstitial lung disease and pulmonary hypertension.

Am J Respir Crit Care Med 2025;211:A6436.

American Thoracic Society 2025 International Conference, May 18-21, San Francisco