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United Therapeutics Corporation Announces Full Enrollment of the TETON-PPF Study of Inhaled Treprostinil for the Treatment of Progressive Pulmonary Fibrosis

UTHR
  • United Therapeutics completed full enrollment of the TETON-PPF study in August 2026, enrolling 754 patients to evaluate nebulized Tyvaso for progressive pulmonary fibrosis (PPF).
  • Approximately 200,000 patients in the United States have PPF, a condition with limited current treatment options.
  • Topline data from the TETON-PPF study is expected in the second half of 2027.
  • The study is a 52-week, randomized, double-blind, placebo-controlled phase 3 trial where subjects receive either nebulized Tyvaso or a placebo.
  • Participants start with three breaths four times daily and titrate up to a target dose of 12 breaths four times daily.
  • The primary endpoint is the change in absolute forced vital capacity (FVC) from baseline to week 52.
  • Secondary endpoints include time to first clinical worsening, time to first acute exacerbation of ILD, overall survival, change in percent predicted FVC, King's Brief Interstitial Lung Disease questionnaire scores, and change in diffusing capacity of the lungs for carbon monoxide.
  • If successful, United Therapeutics plans to submit a supplemental New Drug Application (sNDA) to the FDA to add PPF to the approved indications for nebulized Tyvaso.
  • The company is also seeking priority review for an sNDA submitted in June 2026 to add idiopathic pulmonary fibrosis (IPF) to the labeled indications for nebulized Tyvaso.
  • Nebulized Tyvaso is currently approved for pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD) but remains investigational for PPF.
  • Tyvaso is a prostacyclin mimetic that acts as a pulmonary and systemic vasodilator, with common adverse reactions including cough, headache, throat irritation, nausea, flushing, syncope, dizziness, and diarrhea.
  • Warnings for Tyvaso include risks of symptomatic hypotension in patients with low systemic arterial pressure, increased bleeding risk due to platelet aggregation inhibition, and potential acute bronchospasm in patients with asthma or COPD.