A Study to Assess Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Hypertension Associated With Pulmonary Fibrosis

Study Overview
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Brief Summary
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A randomized, double-blind, placebo-controlled dose escalation study to assess the safety and efficacy of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary fibrosis on long term oxygen therapy.

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

Official Title
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A Randomized, Double-Blind, Placebo-Controlled Dose Escalation Clinical Study to Assess the Safety and Efficacy of Pulsed Inhaled Nitric Oxide (iNO) in Subjects With Pulmonary Hypertension Associated With Pulmonary Fibrosis on Long Term Oxygen Therapy (Part 1 and Part 2)

Conditions
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Pulmonary Fibrosis, Pulmonary Hypertension

Intervention / Treatment
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* Combination Product: INOpulse®
* Combination Product: Placebo
* Combination Product: Long Term Follow Up


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 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 one of the following guidelines, as per American Thoracic Society (AGS) / European Respiratory Society (ERS) / Japanese Respiratory Society (JRS) / Latin American Thoracic Association (ALAT): Major idiopathic interstitial pneumonias (IIPs) 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 Have been using oxygen therapy by nasal cannula for at least 4 weeks 6-Minute Walk Distance (6MWD) ≥ 100 meters and ≤ 450 meters at screening and Baseline/Randomization visits World Health Organization (WHO) Functional Class II-IV Forced Vital Capacity ≥ 40% predicated within the last 6 months prior to the screening run-in period Age between 18 and 85 years (inclusive) Exclusion Criteria: Pregnant or breastfeeding females at Screening 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 local radiologist/Investigator

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 85 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Quadruple


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Inhaled Nitric Oxide (iNO)<br>Pulsed inhaled iNO30, 45 and 75 mcg/kg Ideal Body Weight (IBW)/hour (hr) | Combination Product: INOpulse®<br>* Patients will be treated by means of an INOpulse® device using an INOpulse® nasal cannula<br>|
| Placebo Comparator: Placebo<br>Pulsed inhaled N2, 99.999% gas | Combination Product: Placebo<br>* Patients will be treated by means of an INOpulse® device using an INOpulse® nasal cannula<br>|
| Active Comparator: Long Term Follow Up<br>Pulsed inhaled iNO30, 45 or 75 mcg/kg IBW/hr | Combination Product: Long Term Follow Up<br>* Patients will be treated by means of an INOpulse® device using an INOpulse® nasal cannula<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Efficacy of escalating doses of iNO in patient's life participation as assessed in minutes per day of moderate to vigorous activity. | Part 1 - Blinded Treatment Period | Change from baseline to week 8 or 16 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary Fibrosis (PF), Pulmonary Hypertension, Inhaled Nitric Oxide, iNO, Long Term Oxygen Therapy, Oxygen