Clinical Trial Finder

Inclusion Criteria:

1. Signed Informed Consent 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): Major IIPs (idiopathic interstitial pneumonias) diagnosis or suspected as one of the following:

• - 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 Chronic hypersensitivity pneumonitis 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 screening the screening run-in period. 8. 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. 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. Subjects must have completed at least 1 week of activity monitoring prior to the Baseline/Randomization visit. 11. Age between 18 and 85 years (inclusive) 12. Subject should be clinically stable for at least 4 weeks prior to Baseline/Randomization in the opinion of the Principal Investigator.

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 prior to Baseline/Randomization 3. Use of any PAH-specific medications regardless of reason for use 4. Acute or chronic physical impairment (other than dyspnea due to PF) 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 any connective tissue disease with FVC > 60% unless there is evidence of moderate to severe fibrosis on CT scan in the opinion of the 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 Investigator

A phase 2b, randomized, double-blind, placebo-controlled dose escalation clinical study to assess the safety and efficacy of pulsed, inhaled nitric oxide (iNO) versus placebo in subjects with and without pulmonary hypertension associated with pulmonary fibrosis on long term oxygen therapy (Part 1 and Part 2).

Arms

Experimental: Cohort 1

Part 1: iNO or placebo 30 mcg/kg IBW/hr for a 1 week run-in period and 8 week treatment period Part 2: Open Label Therapy iNO30 mcg/kg IBW/hr for 8 weeks, iNO45 mcg/kg IBW/hr for 8 weeks, iNO75 mg/kg IBW/hr for 8 weeks followed by long term open label therapy.

Experimental: Cohort 2

Part 1: iNO or placebo 45 mcg/kg IBW/hr for 1 week run-in period and a 16 week treatment period Part 2: Open Label Therapy iNO45 mcg/kg IBW/hr for 8 weeks, iNO 75 mcg/kg/hr for 8 weeks followed by long term open label therapy

Experimental: Cohort 3

Part 1: iNO or placebo 75 mcg/kg IBW/hr for 1 week run-in period and a 16 week treatment period Part 2: Open Label Therapy iNO75 mcg/kg IBW/hr for 8 weeks followed by long term open label therapy

Interventions

Drug: - iNO

iNO30 mcg/kg IBW/hr, iNO45 mcg/kg IBW/hr, iNO75 mcg/kg IBW/hr

Drug: - Placebo

Placebo