Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Progressive Pulmonary Fibrosis (TETON-PPF)

Inclusion Criteria:

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

2. Subject is ≥18 years of age, inclusive, at the time of signing informed consent.

3. Subject has radiological evidence of pulmonary fibrosis of >10% extent on an HRCTscan in the previous 12 months (confirmed by central review).

4. Subject has a diagnosis of PPF (other than IPF) that fulfills at least 1 of thefollowing criteria for progression within 24 months of screening despite standardtreatment of ILD, as assessed by the Investigator:

5. Clinically significant decline in % predicted FVC based on ≥10% relativedecline

6. Marginal decline in % predicted FVC based on ≥5% to <10% relative declinecombined with worsening of respiratory symptoms

7. Marginal decline in % predicted FVC based on ≥5% to <10% relative declinecombined with increasing extent of fibrotic changes on chest imaging

8. Worsening of respiratory symptoms as well as increasing extent of fibroticchanges on chest imaging

9. FVC ≥45% predicted at Screening (confirmed by central review).

10. Subjects must be on 1 of the following:

11. On nintedanib or pirfenidone for ≥90 days prior to Baseline and in theInvestigator's opinion, are planning to continue treatment through the study

12. Not on treatment with nintedanib or pirfenidone for ≥90 days prior to Baselineand in the Investigator's opinion, not planning to initiate either treatmentduring the study. Concomitant use of both nintedanib and pirfenidone is not permitted.

13. Subjects treated with immunosuppressive agents (eg, mycophenolate, methotrexate,azathioprine, oral corticosteroids, rituximab) need to be on treatment for at least 120 days prior to Baseline and, in the Investigator's clinical opinion, must berefractory to treatment.

14. Women of childbearing potential must be non-pregnant (as confirmed by a urinepregnancy test at Screening and Baseline) and non-lactating, and will agree to do 1of the following:

15. Abstain from intercourse (when it is in line with their preferred and usuallifestyle)

16. Use 2 medically acceptable, highly effective forms of contraception for theduration of the study, and at least 30 days after discontinuing study drug. i. Medically acceptable, highly effective forms of contraception can includeapproved hormonal contraceptives (oral, injectable, and implantable) and barriermethods (such as a condom or diaphragm) when used with a spermicide. Women who are successfully sterilized (including hysterectomy, bilateralsalpingectomy, or bilateral oophorectomy) or postmenopausal (defined as amenorrheafor at least 12 consecutive months) are not considered to be of reproductivepotential.

17. Males with a partner of childbearing potential must agree to use a condom for theduration of treatment and for at least 48 hours after discontinuing study drug.

18. 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 (forced expiratory volume in 1second/FVC <0.70 at Screening) or greater extent of emphysema than fibrosis on HRCT (confirmed by central review).

3. Subject has a diagnosis of IPF.

4. Subject has shown intolerance or significant lack of efficacy to a prostacyclin orprostacyclin analogue that resulted in discontinuation or inability to effectivelytitrate that therapy.

5. 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 prior to anystudy-related efficacy assessments.

6. Subject is receiving >10 L/min of oxygen supplementation by any mode of delivery atrest at Baseline.

7. Exacerbation of ILD 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 ILDor a pulmonary or upper respiratory infection, subjects must have been dischargedmore than 90 days prior to Baseline to be eligible.

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

9. 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.

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

11. 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.

12. In the opinion of the Investigator, life expectancy <12 months due to ILD or aconcomitant illness.