Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis

Inclusion Criteria:

1. Subject gives voluntary informed consent to participate in the study.

2. Subject is ≥40 years of age, inclusive, at the time of signing informed consent.

3. The subject has a diagnosis of IPF based on the 2018 ATS/ERS/JRS/ALAT ClinicalPractice Guideline (Raghu 2018) and confirmed by central review of high-resolutioncomputed tomography (HRCT) (performed within the previous 12 months), and ifavailable, surgical lung biopsy.

4. FVC ≥45% predicted at Screening.

5. Subjects on pirfenidone or nintedanib must be on a stable and optimized dose for ≥30days prior to Baseline. Concomitant use of both pirfenidone and nintedanib is notpermitted.

6. Women of childbearing potential must be non-pregnant (as confirmed by a urinepregnancy test at Screening and Baseline) and non-lactating, and will abstain fromintercourse (when it is in line with their preferred and usual lifestyle) or use 2medically acceptable, highly effective forms of contraception for the duration ofthe study, and at least 30 days after discontinuing study drug.

7. Males with a partner of childbearing potential must use a condom for the duration oftreatment and for at least 48 hours after discontinuing study drug.

8. In the opinion of the Investigator, the subject is able to communicate effectivelywith study personnel, and is considered reliable, willing, and likely to becooperative with protocol requirements, including attending all study visits.

Exclusion Criteria:

1. Subject is pregnant or lactating.

2. Subject has primary obstructive airway physiology: FEV1/FVC <0.70 at Screening.

3. The subject has shown intolerance or significant lack of efficacy to a prostacyclinor prostacyclin analogue that resulted in discontinuation or inability toeffectively titrate that therapy.

4. The subject has received any PAH-approved therapy, including prostacyclin therapy (epoprostenol, treprostinil, iloprost, or beraprost; except for acute vasoreactivitytesting), IP receptor agonists (selexipag), endothelin receptor antagonists,phosphodiesterase type 5 inhibitors (PDE5-Is), or soluble guanylate cyclasestimulators within 60 days prior to Baseline. As needed use of a PDE5-I for erectiledysfunction is permitted, provided no doses are taken within 48 hours of anystudy-related efficacy assessments.

5. Use of any of the following medications: azathioprine (AZA), cyclosporine,mycophenolate mofetil, tacrolimus, oral corticosteroids (OCS) >20 mg/day or thecombination of OCS+AZA+N-acetylcysteine within 30 days prior to Baseline;cyclophosphamide within 60 days prior to Baseline; or rituximab within 6 monthsprior to Baseline.

6. The subject is receiving >10 L/min of oxygen supplementation by any mode of deliveryat rest at Baseline.

7. Exacerbation of IPF or active pulmonary or upper respiratory infection within 30days prior to Baseline. Subjects must have completed any antibiotic or steroidregimens for treatment of the infection or acute exacerbation more than 30 daysprior to Baseline to be eligible. If hospitalized for an acute exacerbation of IPFor a pulmonary or upper respiratory infection, subjects must have been dischargedmore than 90 days prior to Baseline to be eligible.

8. Uncontrolled cardiac disease, defined as myocardial infarction within 6 months priorto Baseline or unstable angina within 30 days prior to Baseline.

9. In the opinion of the Investigator, the subject has any condition that wouldinterfere with the interpretation of study assessments or would impair studyparticipation or cooperation.

10. Use of any other investigational drug/device or participation in any investigationalstudy in which the subject received a medical intervention (ie, procedure, device,medication/supplement) within 30 days prior to Screening. Subjects participating innon-interventional, observational, or registry studies are eligible.

11. Life expectancy <6 months due to IPF or a concomitant illness.

12. Acute pulmonary embolism within 90 days prior to Baseline.