Combined results of the TETON-1 and TETON-2 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis
Abstract
BACKGROUND: Inhaled treprostinil has been shown to slow the rate of loss of lung function in IPF patients in two independent studies. A detailed analysis of the fully integrated results of both studies will provide more precise and robust estimates of the drug's efficacy in IPF. METHODS: TETON-1 and TETON-2 were two replicate phase 3 double-blind trials, where patients with IPF were randomized to inhaled treprostinil or placebo. The primary endpoint was change in absolute forced vital capacity (FVC) at week 52. Secondary outcomes were time to clinical worsening, time to IPF exacerbation, survival, percent predicted FVC (ppFVC), quality of life and percent predicted diffusing capacity (ppDLCO) by 52 weeks. RESULTS: There were 1191 patients who underwent randomization and received at least one dose of treprostinil (597 patients) or placebo (594 patients) across both trials. Median change from baseline in FVC was -45.4 ml (95% CI, -73.8 to -23.1) in the treprostinil group and -161.7 ml (95% CI, -194.5 to -134.1) in the placebo group (between-group difference 111.8 ml (95% CI, 79.7 to 144.0, p < 0.0001) at 52 weeks. Treprostinil demonstrated superiority to placebo in five of six secondary efficacy endpoints including: time to first clinical worsening, time to first IPF exacerbation, change from baseline in the K-BILD, ppDLCO, and ppFVC. There were 41 (6.9%) deaths in the inhaled treprostinil group versus 56 (9.4%) deaths in the placebo group. Cough was the most common adverse event. CONCLUSIONS: Inhaled treprostinil slowed the rate of lung function loss, delayed clinical worsening and IPF exacerbations, and demonstrated benefits in DLCO and quality of life over 52 weeks. The results support the use of inhaled treprostinil in patients with IPF.(Funded by United Therapeutics Corp; ClinicalTrials.gov number, NCT04708782 for TETON-1 and NCT05255991 for TETON-2).
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BibTeX
@article{nathan2026combined,
title = {{Combined results of the TETON-1 and TETON-2 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis}},
author = {Steven D Nathan and Peter Smith and Chunqin Deng and Jeffrey Golden and Martine Reynaud-Gaubert and Granthem Farr and Benjamin Bregman and Peter F Crossno and Sergio Harari and Nishant Gupta and Stéphane Jouneau and Yochai Adir and Maria Molina-Molina and Natalie Breytenbach and Leigh Peterson and Heidi Bell and Emilia Zywot and Youlan Rao and Kevin R. Flaherty and Vincent Cottin and Juergen Behr},
journal = {American Journal of Respiratory and Critical Care Medicine},
year = {2026},
month = nov,
doi = {10.1093/ajrccm/aamag507},
url = {https://hal.science/hal-05851999v1},
note = {CC BY},
}
RIS
TY - JOUR
TI - Combined results of the TETON-1 and TETON-2 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis
AU - Steven D Nathan
AU - Peter Smith
AU - Chunqin Deng
AU - Jeffrey Golden
AU - Martine Reynaud-Gaubert
AU - Granthem Farr
AU - Benjamin Bregman
AU - Peter F Crossno
AU - Sergio Harari
AU - Nishant Gupta
AU - Stéphane Jouneau
AU - Yochai Adir
AU - Maria Molina-Molina
AU - Natalie Breytenbach
AU - Leigh Peterson
AU - Heidi Bell
AU - Emilia Zywot
AU - Youlan Rao
AU - Kevin R. Flaherty
AU - Vincent Cottin
AU - Juergen Behr
PY - 2026
DA - 2026/11/04
JO - American Journal of Respiratory and Critical Care Medicine
DO - 10.1093/ajrccm/aamag507
UR - https://hal.science/hal-05851999v1
AB - BACKGROUND: Inhaled treprostinil has been shown to slow the rate of loss of lung function in IPF patients in two independent studies. A detailed analysis of the fully integrated results of both studies will provide more precise and robust estimates of the drug's efficacy in IPF. METHODS: TETON-1 and TETON-2 were two replicate phase 3 double-blind trials, where patients with IPF were randomized to inhaled treprostinil or placebo. The primary endpoint was change in absolute forced vital capacity (FVC) at week 52. Secondary outcomes were time to clinical worsening, time to IPF exacerbation, survival, percent predicted FVC (ppFVC), quality of life and percent predicted diffusing capacity (ppDLCO) by 52 weeks. RESULTS: There were 1191 patients who underwent randomization and received at least one dose of treprostinil (597 patients) or placebo (594 patients) across both trials. Median change from baseline in FVC was -45.4 ml (95% CI, -73.8 to -23.1) in the treprostinil group and -161.7 ml (95% CI, -194.5 to -134.1) in the placebo group (between-group difference 111.8 ml (95% CI, 79.7 to 144.0, p < 0.0001) at 52 weeks. Treprostinil demonstrated superiority to placebo in five of six secondary efficacy endpoints including: time to first clinical worsening, time to first IPF exacerbation, change from baseline in the K-BILD, ppDLCO, and ppFVC. There were 41 (6.9%) deaths in the inhaled treprostinil group versus 56 (9.4%) deaths in the placebo group. Cough was the most common adverse event. CONCLUSIONS: Inhaled treprostinil slowed the rate of lung function loss, delayed clinical worsening and IPF exacerbations, and demonstrated benefits in DLCO and quality of life over 52 weeks. The results support the use of inhaled treprostinil in patients with IPF.(Funded by United Therapeutics Corp; ClinicalTrials.gov number, NCT04708782 for TETON-1 and NCT05255991 for TETON-2).
SN - 1073-449X
SN - 1535-4970
ER -