This is a Phase 3, randomized, double-blind, placebo-controlled, multinational, efficacy and safety study of subjects with PPF treated with inhaled treprostinil over a 52-week period. (TETON-IPF)

Study Population

Approximately 698 subjects will be randomly assigned to study intervention at approximately 150 centers globally.

PPF is a lung disease that occurs when lung tissue becomes progressively damaged and scarred. This thickened, stiff lung tissue makes it more difficult for lungs to work properly, which causes shortness of breath.

Treprostinil belongs to a group of drugs known as vasodilators. Vasodilators help to reduce the pressure in the arteries and widen the blood vessels in the lungs and other parts of the body. This helps the blood along with oxygen to flow more easily into your lungs. Treprostinil may also reduce lung scarring.

Eligible subjects must be:

≥18 years of age

have a diagnosis of Progressive Pulmonary Fibrosis (other than idiopathic pulmonary fibrosis) with >10% extent of fibrosis on HRCT

have a FVC ≥45% predicted

Subjects must be either: a) on nintedanib or pirfenidone for ≥90 days OR

b) not on nintedanib or pirfenidone

Subjects with primary obstructive airway physiology (forced expiratory volume in 1 second/FVC <0.70), those on >10 L/min of supplemental oxygen at rest at Baseline, and women who are pregnant or lactating will not be eligible to participate in the study.

Study visits

9 onsite visits over 52 weeks

The study comprises of a Screening period of up to 6 weeks, Treatment period of 52 weeks, and an End of Study visit

The Treatment period has 6 onsite vists, with weekly phone contact for the first 4 months, then monthly phone calls between onsite visits until end of study.

Study Requirements

Medication is taken via nebuliser 4 times/day

Dosing diary to record medication administration times

Questionnaires, ECG, Blood test (non-fasting), Spirometry at all onsite vsits.

Monthly phone contact with site

Clinical Trials Registry:

https://clinicaltrials.gov/study/NCT05943535