Principal Investigator: Mary  Scholand
Keywords: Pulmonary , Pulmonary Hypertension , Nitric Oxide , IPF , Idiopathic Pulmonary Fibrosis , UIP , Usual Interstitial Pneumonia , ILD , Interstitial Lung Disease Department: Pulmonary
IRB Number: 00106356
Specialty: Pulmonary
Sub Specialties: Pulmonary Fibrosis
Recruitment Status: Active, not recruiting

Contact Information

Cassie Larsen

Brief Summary

Part 1: Blinded Treatment Period
Primary Objective: The primary objective in this study is to evaluate the efficacy and optimal
dose of iNO in subjects with PH-PF currently receiving treatment with LTOT.

Part 2: Open Label Period
Primary Objective: To evaluate the long term safety and tolerability of INOpulse in study participants with fILD currently receiving treatment with oxygen therapy.

Inclusion Criteria

1. Signed Informed Consent Form (and assent as appropriate) prior to the initiation of any study
mandated procedures or assessments.
2. Diagnosed with pulmonary fibrosis by high resolution CT scan performed in the 6 months
prior to screening associated with one of the following conditions and confirmed using
guidelines, as per American Thoracic Society (ATS) / European Respiratory Society (ERS) /
Japanese Respiratory Society (JRS) / Latin American Thoracic Association (ALAT):
2.1 Major IIPs (idiopathic interstitial pneumonias) diagnosis or suspected as one of the
Idiopathic pulmonary fibrosis
Idiopathic nonspecific interstitial pneumonia
Respiratory bronchiolitis-interstitial lung disease
Desquamative interstitial pneumonia
Cryptogenic organizing pneumonia
Acute interstitial pneumonia
Rare IIPs diagnosis by one of the following:
Idiopathic lymphoid interstitial pneumonia
Idiopathic pleuroparenchymal fibroelastosis
Unclassifiable idiopathic interstitial pneumonias
2.2 Chronic hypersensitivity pneumonitis
2.3 Occupational lung disease

3. At least 50% of the subjects will have confirmed intermediate or high probability of
pulmonary hypertension as determined by echocardiography according to the 2015 ESC/ERS
Guidelines for Diagnosis and Treatment of Pulmonary Hypertension.
4. Have been using oxygen therapy by nasal cannula for at least 4 weeks prior to the screening
run-in period.
5. 6MWD ≥ 100 meters and ≤ 450 meters prior to randomization.
6. WHO Functional Class II-IV
7. Forced Vital Capacity ≥ 40% predicted within last 6 months prior to the screening run-in
8. Willingness to use INOpulse delivery device for at least 12 hours per day
9. Female subjects of childbearing potential must have a negative pre-treatment pregnancy test
(serum or urine). All female subjects should take adequate precaution to avoid pregnancy.
10. Have completed at least 1 week of activity monitoring at time of Baseline/Randomization
11. For at least 1 week prior to Baseline/Randomization, subjects must demonstrate the ability to
consistently use the device greater than 12 hrs/day in the opinion of the Investigator.
12. Age between 18 and 85 years (inclusive). 
13. Subject should be clinically stable for at least 4 weeks in the opinion of the Principal

Part 2: Open Label Period

1. All study participants must have completed Part 1 – Blinded Treatment Period study assessments. For Cohort 3 only, study participants must have been compliant with the INOpulse device use.

2. In the opinion of the investigator, the subject would benefit from continued therapy with iNO.

Exclusion Criteria

1. Demonstrate symptomatic rebound defined as significant cardiopulmonary instability, such
as systemic arterial oxygen desaturation, hypoxemia, bradycardia, tachycardia, systemic
hypotension, shortness of breath, near-syncope, and syncope, occurring within 1 hour of
acute iNO during rebound testing
2. Episodes of disease worsening within 1 month of Baseline/Randomization
3.Use of any type of PAH-specific medications regardless of reason for use 
4. Acute or chronic physical impairment (other than dyspnea due to IPF) that would limit the
ability to comply with study procedures or adherence to therapy (i.e., 6MWT), including
carrying and wearing the pulsed delivery device per study protocol, or medical problem(s)
likely to preclude completion of the study
5. Pregnant or breastfeeding females at Screening
6. Administered L-arginine within 1 month prior to Screening
7. The concurrent use of the INOpulse device with a continuous positive airway pressure
(CPAP), bilevel positive airway pressure BPAP, or any other positive pressure device.
8. Use of investigational drugs or devices within 1 month prior to Screening (other than acute
vasodilator testing with iNO)

9. Any underlying medical or psychiatric condition that, in the opinion of the Investigator,
makes the subject an unsuitable candidate for the study including unable to complete 6MWT
10. Any subject who has been enrolled in any previous clinical study with inhaled NO
administered through pulse delivery
11. In the last 6 months prior to screening, evidence of connective tissue disease with FVC > 60% unless there is evidence of moderate to severe fibrosis on CT scan in the local radiologist/Investigator
12.Evidence of clinically significant Combined Pulmonary Fibrosis and Emphysema (CPFE) if > 15% of lung fields by CT scan show evidence of emphysema in the opinion of the local radiologist/Investigator.

Part 2: Open Label Period (All Cohorts)

1. Use of a prostacyclin analogue, guanylate cyclase stimulator or ERA PAH-specific medications regardless of reason for use (use of PDE-5 inhibitors regardless of reason for use is allowed).

2. The concurrent use of the INOpulse device with CPAP/BPAP, or any other positive airway pressure device.