Abstract Rationale Progressive pulmonary fibrosis (PPF) consists of patients with interstitial lung disease (ILD) who exhibit a similar disease course to idiopathic pulmonary fibrosis (IPF). The recently completed RIN-PF-303 study in patients with IPF demonstrated the superiority of inhaled treprostinil over placebo for the change in absolute FVC over 52 weeks. Inhaled treprostinil is being studied in the TETON-PPF trial of patients with PPF based upon preclinical evidence of an antifibrotic mechanism and supportive clinical observation. Methods TETON-PPF is a 52-week, phase 3 study enrolling 698 subjects with a diagnosis of PPF and 10% fibrosis on chest computed tomography. Background use of pirfenidone or nintedanib is allowed. The primary endpoint is change in absolute FVC at week 52. Key secondary endpoints include time to clinical worsening, time to acute exacerbation of ILD, and overall survival. Results Enrollment in this study is ongoing in approximately 16 countries globally until target randomization is reached. Conclusions The TETON-PPF Phase 3 clinical trial aims to investigate inhaled treprostinil as a potential treatment option for patients with PPF. The patients recruited thus far have moderate restrictive disease and about half are on antifibrotic therapy. This abstract is funded by: United Therapeutics Corporation
Nathan et al. (Fri,) studied this question.