A Study to Assess Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Hypertension Associated With Pulmonary Fibrosis
Pulmonary Fibrosis, Pulmonary Hypertension
About this trial
This is an interventional treatment trial for Pulmonary Fibrosis focused on measuring Pulmonary Fibrosis (PF), Pulmonary Hypertension, Inhaled Nitric Oxide, iNO, Long Term Oxygen Therapy, Oxygen
Eligibility Criteria
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
Sites / Locations
- Arizona Pulmonary Specialists
- University of California
- University of California Davis Health
- University of Colorado Hospital
- University of Miami
- Avanza Medical Research Center
- Emory University
- Piedmont Healthcare Pulmonary & Critical Care Research
- Loyola University
- Norton Pulmonary Specialists
- University of Michigan
- The Lung Research Center (St. Luke's)
- The University of North Carolina at Chapel Hill
- University of Cincinnati College of Medicine
- Thomas Jefferson University Korman Respiratory Institute
- Temple University Hospital
- Medical University of South Carolina
- Vanderbilt University Medical Center
- University of Texas Southwestern Medical Center
- University of Utah Health Sciences
- Inova Heart and Vascular Institute Advanced Lung Disease Clinic
- Pulmonary Associates of Richmond
- University of Washington Medical Center
Arms of the Study
Arm 1
Arm 2
Arm 3
Active Comparator
Placebo Comparator
Active Comparator
Inhaled Nitric Oxide (iNO)
Placebo
Long Term Follow Up
Pulsed inhaled iNO30, 45 and 75 mcg/kg Ideal Body Weight (IBW)/hour (hr)
Pulsed inhaled N2, 99.999% gas
Pulsed inhaled iNO30, 45 or 75 mcg/kg IBW/hr