{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T17:01:35Z","timestamp":1790182895081,"version":"4.1.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T00:00:00Z","timestamp":1790121600000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>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 \u221245.4\u2009ml (95% CI, -73.8 to -23.1) in the treprostinil group and \u2212161.7\u2009ml (95% CI, -194.5 to -134.1) in the placebo group (between-group difference 111.8\u2009ml (95% CI, 79.7 to 144.0, p\u2009&amp;lt;\u20090.0001) 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                    <jats:p>(Funded by United Therapeutics Corp; ClinicalTrials.gov number, NCT04708782 for TETON-1 and NCT05255991 for TETON-2)<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ajrccm\/aamag507","type":"journal-article","created":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:39:50Z","timestamp":1790077190000},"source":"Crossref","is-referenced-by-count":0,"title":["Combined results of the TETON-1 and TETON-2 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis"],"prefix":"10.1093","author":[{"given":"Steven D","family":"Nathan","sequence":"first","affiliation":[{"name":"Inova Fairfax Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Smith","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunqin","family":"Deng","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey","family":"Golden","sequence":"additional","affiliation":[{"name":"University of California San Francisco , School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Reynaud-Gaubert","sequence":"additional","affiliation":[{"name":"H\u00f4pital Universitaire de Marseille Nord; Aix Marseille Universit\u00e9 C2VN -INSERM1263 - INRAE1260 Centre de Comp\u00e9tences des Maladies Pulmonaires Rares, , Marseille;","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Granthem","family":"Farr","sequence":"additional","affiliation":[{"name":"The Oregon Clinic , Portland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Bregman","sequence":"additional","affiliation":[{"name":"Southeastern Research Center , Winston Salem, NC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter F","family":"Crossno","sequence":"additional","affiliation":[{"name":"Intermountain Healthcare , Murray Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergio","family":"Harari","sequence":"additional","affiliation":[{"name":"University of Milan Department of Clinical Sciences and Community Health, and Divsion of Pulmonary Diseases, , Milan,","place":["Italy"]},{"name":"MultiMedica IRCCS Department of Clinical Sciences and Community Health, and Divsion of Pulmonary Diseases, , Milan,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nishant","family":"Gupta","sequence":"additional","affiliation":[{"name":"University of Cincinnati Division of Pulmonary, Critical Care and Sleep Medicine,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Jouneau","sequence":"additional","affiliation":[{"name":"CHU de Rennes Centre de R\u00e9f\u00e9rence pour les Maladies Rares Pulmonaires , Rennes Cedex,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yochai","family":"Adir","sequence":"additional","affiliation":[{"name":"Lady Davis Carmel Medical Center ,","place":["Israel"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Molina-Molina","sequence":"additional","affiliation":[{"name":"Hospital Universitario de Bellvitge, IDIBELL, University of Barcelona Respiratory Department, ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalie","family":"Breytenbach","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leigh","family":"Peterson","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Bell","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilia","family":"Zywot","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youlan","family":"Rao","sequence":"additional","affiliation":[{"name":"United Therapeutics Corporation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin R","family":"Flaherty","sequence":"additional","affiliation":[{"name":"University of Michigan Michigan Medicine, , Ann Arbor"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Claude Bernard Lyon 1, Hospices Civils de Lyon, Centre de r\u00e9f\u00e9rence des maladies pulmonaires rares , Service de pneumologie, , , , F-69000 Lyon,","place":["France"]},{"name":"ERN-LUNG , Service de pneumologie, , , , F-69000 Lyon,","place":["France"]},{"name":"INRAE , Service de pneumologie, , , , F-69000 Lyon,","place":["France"]},{"name":"UMR754 , Service de pneumologie, , , , F-69000 Lyon,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juergen","family":"Behr","sequence":"additional","affiliation":[{"name":"LMU Munich, German Center for Lung Research (DZL) Department of Medicine V, LMU University Hospital, Comprehensive Pneumology Center, , Munich,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the TETON-1 and TETON-2 Trial Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,9,23]]},"container-title":["American Journal of Respiratory and Critical Care 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