A Study to Assess Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis at Risk for Pulmonary Hypertension (REBUILD)
Pulmonary Fibrosis, Pulmonary Hypertension
![Bellerophon Pulse Technologies](https://trials-media.s3.amazonaws.com/orgs/bellerophon-pulse-technologies.jpeg)
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, REBUILD, INOpulse, pulsed inhaled nitric oxide, fILD, fibrotic interstitial lung disease, portable pulsed inhaled nitric oxide
Eligibility Criteria
Inclusion criteria:
A 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
- Connective Tissue Disease associated with IPF (CTD-ILD)
- Interstitial Pneumonia with Autoimmune Features (IPAF)
- Have been using oxygen therapy by nasal cannula for at least 4 weeks (including use limited to exertion)
- 6MWD ≥ 100 meters and ≤ 400 meters at screening and Baseline/Randomization visits.
- World Health Organization (WHO) Functional Class II-IV
- Forced Vital Capacity ≥ 40% predicted within the 60 day Screening period
- Age between 18 and 80 years (inclusive) at screening
Exclusion criteria:
- For women of child-bearing potential: Pregnant or breastfeeding females at Screening, or planning to get pregnant, or unwilling to use appropriate contraception if sexually active to avoid pregnancy during the study and for at least 30 days after discontinuation of the study drug.
- Heart failure with reduced ejection fraction (HFrEF; systolic heart failure) with an ejection fraction of < 40%; or severe HF with preserved EF (HFpEF; diastolic HF)
- History of sarcoidosis
- History of chronic thrombo-embolic pulmonary hypertension (CTEPH; WHO group 4 PH)
- Smoking within 3 months of Screening and/or unwilling to avoid smoking throughout the study
- Body mass index (BMI) >40 kg/m2 at screening
- Intermittent uncontrolled atrial fibrillation, in the opinion of the Principal Investigator
- Known severe hepatic impairment, in the opinion of the Principal Investigator
- Known severe renal impairment (Chronic Kidney Disease Epidemiology Collaboration (CKDEPI) 2009 equation [Levy 2009] calculated creatinine clearance <30 ml/min) at screening
Sites / Locations
- University of Alabama
- Banner - University Medical Center Arizona
- Arizona Pulmonary Specialists, Ltd.
- University of California San Francisco
- University of California
- Amicis Research
- Paloma Medical Group
- University of California Davis Health
- Stanford University Medical Center
- UC Denver Anschutz Medical Center
- National Jewish
- Georgetown University
- St. Francis Sleep Allergy and Lung Institute
- Advanced Pulmonary Research Institute
- University of Miami
- Avanza Medical Research Center
- University of South Florida
- Cleveland Clinic
- Emory University
- Northside Hospital
- Piedmont Healthcare
- Northwestern
- North Shore University Hospital
- Loyola University
- Southern Illinois University
- Ascension St. Vincent Hospital
- Norton Pulmonary Specialists
- University of Louisville
- Johns Hopkins Hospital
- Tufts Medical Center
- Brigham and Women's Hospital
- University of Michigan
- Henry Ford Hospital
- The Lung Research Center, LLC
- Creighton University
- Atlantic Health Respiratory Institute
- NYU Langone
- Mount Sinai Hospital
- Weill Cornell Medical Center
- Stony Brook University Hospital
- The University of North Carolina at Chapel Hill Marsico Clinical Research Center
- Pulmonix, LLC/LeBauer Cone Health
- University of Cincinnati
- University Hospitals
- The Ohio State University
- The Oregon Clinic
- Thomas Jefferson University Korman Respiratory Institute
- University of Pennsylvania
- Temple University
- University of Pittsburgh
- AnMed Health
- Medical University of South Carolina
- Vanderbilt University Medical Center
- Baylor University Medical Center
- UT Southwestern Medical Center
- University of Texas Health Science Center at Houston
- Metroplex Pulmonary and Sleep
- University of Utah Health Sciences
- Inova Heart and Vascular Institute Advanced Lung Disease Clinic
- Pulmonary Associates of Richmond
- University of Washington Medical Center
- University of British Columbia
- Queen's University/Hotel Dieu Hospital
- London Health Sciences Centre
Arms of the Study
Arm 1
Arm 2
Arm 3
Active Comparator
Placebo Comparator
Other
Inhaled Nitric Oxide (iNO)
Placebo
Open Label Extension
Pulsed inhaled iNO 45 mcg/kg Ideal Body Weight (IBW)/hour (hr)
Pulsed inhaled N2, 99.999% gas
Pulsed inhaled iNO 45 mcg/kg IBW/hr