TETON is a Phase 3 program consisting of one or more Phase 3 studies of Tyvaso® in subjects with various forms of chronic fibrosing interstitial lung diseases, which includes patients with idiopathic interstitial pneumonias, chronic hypersensitivity pneumonitis, and environmental/occupational lung disease. The first TETON study will enroll subjects with idiopathic pulmonary fibrosis (IPF). The primary endpoint of this study is planned to be the change in absolute forced vital capacity (FVC) from baseline to week 52.
This program was prompted by data from the INCREASE study, which demonstrated improvements in parameters of lung function in pulmonary hypertension patients with fibrotic lung disease, including absolute FVC, and reduced exacerbations of underlying lung disease. Specifically, in the INCREASE study, treatment with inhaled treprostinil resulted in significant improvements in percent predicted FVC at Weeks 8 and 16, with subjects having underlying etiologies of IIP (Week 8: 1.95%, p=0.0373 and Week 16: 2.88%; p=0.0096) and idiopathic pulmonary fibrosis (Week 8: 2.543%; p=0.0380 and Week 16: 3.504%; p=0.0147) showing greater improvement. Consistent positive effects were also observed in patients with CHP and environmental/occupational lung disease. These data points, combined with substantial preclinical evidence of antifibrotic activity of treprostinil, suggest that Tyvaso may offer a treatment option for patients with fibrotic lung disease.