A Randomized, Double-blind, Placebo-controlled, Multinational, Phase 3 Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects with Progressive Pulmonary Fibrosis (TETON-PPF)

The purpose of this research study is to see if a drug called inhaled treprostinil will improve lung function in people with Progressive Pulmonary Fibrosis (PPF)

For more information, visit: https://clinicaltrials.gov/study/NCT05943535

Inova Fairfax Medical Center 
3300 Gallows Rd  
Falls Church, VA, 22042

A subject is eligible for inclusion in this study if all the following criteria apply:

• Subject is ≥18 years of age, inclusive, at the time of signing informed consent.
• Subject has radiological evidence of pulmonary fibrosis of >10% extent on an HRCT scan in the previous 12 months (confirmed by central review).
• Subject has a diagnosis of PPF (other than IPF) that fulfills at least 1 of the following criteria for progression within 24 months ofscreening despite standard treatment of ILD, as assessed by the Investigator:
  • Clinically significant decline in % predicted FVC based on ≥10% relative decline.
  • Marginal decline in % predicted FVC based on ≥5% to <10% relative decline combined with worsening of respiratory symptoms.
  • Marginal decline in % predicted FVC based on ≥5% to <10% relative decline combined with increasing extent of fibrotic changes on chest imaging.
  • Worsening of respiratory symptoms as well as increasing extent of fibrotic changes on chest imaging.
• FVC ≥45% predicted at Screening (confirmed by central review).
• Subjects must be on 1 of the following:
  • On nintedanib or pirfenidone for ≥90 days prior to Baseline and in the Investigator's opinion, are planning to continue treatment through the study.
  • Not on treatment with nintedanib or pirfenidone for ≥90 days prior to Baseline and in the Investigator's opinion, not planning to initiate either treatment during the study. Concomitant use of both nintedanib and pirfenidone is not permitted.
• In the opinion of the Investigator, the subject is able to communicate effectively with study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements, including attending all study visits

A subject is not eligible for inclusion in this study if any of the following criteria apply:

• Subject has primary obstructive airway physiology (forced expiratory volume in 1 second/FVC <0.70 at Screening) or greater extent of emphysema than fibrosis on HRCT (confirmed by central review).
• Subject has a diagnosis of IPF.
• Subject has received any PAH-approved therapy, including prostacyclin therapy (epoprostenol, treprostinil, iloprost, or beraprost; except for acute vasoreactivity testing), IP receptor agonists (selexipag), endothelin receptor antagonists,  phosphodiesterase type 5 inhibitors (PDE5-Is), or soluble guanylate cyclase stimulators within 60 days prior to Baseline. As needed use of a PDE5-I for erectile dysfunction is
  permitted, provided no doses are taken within 48 hours prior to any study-related efficacy assessments.
• Subject is receiving >10 L/min of oxygen supplementation by any mode of delivery at rest at Baseline.
• Exacerbation of ILD or active pulmonary or upper respiratory infection within 30 days prior to Baseline. Subjects must have completed any antibiotic or steroid regimens for treatment of the infection or acute exacerbation more than 30 days prior to Baseline to be eligible. If hospitalized for an acute exacerbation of ILD or a pulmonary or upper respiratory infection, subjects must have been discharged more than 90 days prior to Baseline to be eligible
• Subject has uncontrolled cardiac disease, defined as myocardial infarction within 6 months prior to Baseline or unstable angina within 30 days prior to Baseline.
• Acute pulmonary embolism within 90 days prior to Baseline.