In general, what is the purpose of the research, how long will you be involved?

You are being asked to be in this research study because you have idiopathic pulmonary fibrosis (IPF).  This means that you have scarring of the lungs from an unknown cause.  The scarring makes it hard for the lungs to get oxygen into the blood, which causes shortness of breath and coughing. 

Your participation in this study is voluntary and will last approximately 58 weeks.  This time includes a Screening Period that could last up to approximately 6 weeks plus a 52-week Treatment Period.

If you choose to participate in this study, you will be required to visit the hospital or clinic on at least 8 separate occasions over an approximate 58-week period.  If needed some visits may be conducted as telehealth study visits.

Procedures will be performed during your scheduled visits such as medical history and demographics, physical exam, viral signs, blood testing, ECG, questionnaires, lung function tests, spirometry, and a CT scan.  

A subject is eligible for inclusion in this study if all the following criteria apply:
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 Clinical
Practice Guideline (Raghu 2018) and confirmed by central review of HRCT (performed
within the previous 12 months) and if available, surgical lung biopsy. HRCT imaging
must be “consistent with UIP,” defined as meeting either criteria A, B, and C; or criteria
A and C; or criteria B and C below:
a. Subpleural and basal predominant honeycombing
b. Subpleural and basal predominant reticular pattern with peripheral traction
bronchiectasis or traction bronchiolectasis
c. Absence of atypical features (eg, predominant ground-glass opacity, nodules,
consolidation, etc). If ground-glass opacity is present, it must be less than the
accompanying reticular pattern.
Subjects with HRCT features deemed indeterminate for IPF (subpleural and basal
predominant, subtle, reticulating pattern of fibrosis) may be considered for inclusion if
coupled with a histopathological pattern of “UIP” or “probable UIP” on surgical lung
biopsy and confirmed by central review.
4. FVC ≥45% predicted at Screening.
5. Subjects on pirfenidone or nintedanib must be on a stable and optimized dose for
≥30 days prior to Baseline. Concomitant use of both pirfenidone and nintedanib is not
permitted.
6. Women of childbearing potential must be non-pregnant (as confirmed by a urine
pregnancy test at Screening and Baseline) and non-lactating, and will do 1 of the
following:
a. Abstain from intercourse (when it is in line with their preferred and usual lifestyle)
b. Use 2 medically acceptable, highly effective forms of contraception for the duration of the study, and at least 30 days after discontinuing study drug. Medically acceptable, highly effective forms of contraception can include approved hormonal contraceptives (oral, injectable, and implantable) and barrier methods (such as a condom or diaphragm) when used with a spermicide.
Women who are successfully sterilized (including hysterectomy, bilateral tubal ligation,
or bilateral oophorectomy) or postmenopausal (defined as amenorrhea for at least
12 consecutive months) are not considered to be of reproductive potential.
7. Males with a partner of childbearing potential must use a condom for the duration of
treatment and for at least 48 hours after discontinuing study drug.
8. In the opinion of the Investigator, the subject is able to communicate effectively with
study personnel, and is considered reliable, willing, and likely to be cooperative with protocol requirements, including attending all study visits.

A subject is not eligible for inclusion in this study if any of the following criteria apply:
1. Subject is pregnant or lactating.
2. Subject has primary obstructive airway physiology.
3. The subject has shown intolerance or significant lack of efficacy to a prostacyclin or
prostacyclin analogue that resulted in discontinuation or inability to effectively titrate that
therapy.
4. The subject has received any PAH-approved therapy, including prostacyclin therapy
(epoprostenol, treprostinil, iloprost, or beraprost; except for acute vasoreactivity testing),
IP receptor agonists (selexipag), endothelin receptor antagonists, phosphodiesterase type
5 inhibitors (PDE5-Is), or soluble guanylate cyclase stimulators within 60 days prior to
Baseline. As needed use of a PDE5-I for erectile dysfunction is permitted, provided no
doses are taken within 48 hours of any study-related efficacy assessments.
5. Use of any of the following medications:
a. Azathioprine (AZA), cyclosporine, mycophenolate mofetil, tacroliumus, oral
corticosteroids (OCS) >20 mg/day or the combination of
OCS+AZA+N-acetylcysteine within 30 days prior to Baseline.
b. Cyclophosphamide within 60 days prior to Baseline
c. Rituximab within 6 months prior to Baseline
6. The subject is receiving >10 L/min of oxygen supplementation by any mode of delivery
at rest at Baseline.
7. Exacerbation of IPF or active pulmonary or upper respiratory infection within 30 days
prior to Baseline. Subjects must have completed any antibiotic or steroid regimens for
treatment of the infection or acute exacerbation more than 30 days prior to Baseline to be
eligible. If hospitalized for an acute exacerbation of IPF or a pulmonary or upper
respiratory infection, subjects must have been discharged more than 90 days prior to
Baseline to be eligible.
8. Uncontrolled cardiac disease, defined as myocardial infarction within 6 months prior to Baseline or unstable angina within 30 days prior to Baseline. In the opinion of the Investigator, the subject has any condition that would interfere with
the interpretation of study assessments or would impair study participation or
cooperation.
10. Use of any other investigational drug/device or participation in any investigational study
in which the subject received a medical intervention (ie, procedure, device,
medication/supplement) within 30 days prior to Screening. Subjects participating in
non-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.