Interstitial lung disease · Antifibrotics in IPF
Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis
| Design | Randomised, double-blind, placebo-controlled trial |
|---|---|
| Treatment | Inhaled treprostinil |
| Control | Placebo |
| Population | 593 patients with IPF, mean age 71.7 years, mean baseline FVC 76.8% predicted, 75.4% on background antifibrotics |
| Follow-up | 52 weeks |
| Primary endpoint | Change in FVC at week 52 |
| Result | Median FVC change -49.9 mL (95% CI -79.2 to -19.5) with treprostinil versus -136.4 mL (-172.5 to -104.0) with placebo. Clinical-worsening events were less frequent with treprostinil. |
| Secondary endpoints | Clinical worsening events, exacerbations, survival |
In patients with IPF, inhaled treprostinil was associated with a smaller decline in FVC and fewer clinical-worsening events than placebo over a period of 52 weeks.
The trial authors, in the published abstract
Repurposes a pulmonary vasodilator as an antifibrotic-adjacent therapy in IPF without requiring pulmonary hypertension, extending the logic of its use in PH-ILD. Like FIBRONEER-IPF, the finding that matters is that it adds to background antifibrotic therapy.
Nathan SD, Smith P, Deng C, et al. Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis. N Engl J Med 2026 Jul 9;395(2):127-137.