A Dose Escalation Study to Assess the Safety and Efficacy of Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis or Sarcoidosis

Study Purpose

A phase 2b, open label study to assess the safety and efficacy of increasing doses of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary fibrosis and sarcoidosis on long term oxygen therapy followed by a long term extension study

Recruitment Criteria

Accepts Healthy Volunteers

Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms

Study Type

An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes.

An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes.

Searching Both is inclusive of interventional and observational studies.

Eligible Ages 18 Years - 85 Years
Gender All
More Inclusion & Exclusion Criteria

Inclusion Criteria:

1. Signed informed consent. 2. A primary diagnosis of sarcoidosis as defined by the ATS/ERS/WASOG statement or pulmonary fibrosis associated with one of the following conditions: 2.1 Major IIPs (idiopathic interstitial pneumonias) diagnosed 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.
2.2 Chronic hypersensitivity pneumonitis. 2.3 Occupational lung disease. 2.4 Connective tissue disease with evidence of significant pulmonary fibrosis. 3. Intermediate or high probability of PH by echocardiogram as assessed by local Radiologist/Investigator, or PH as determined by a right heart catheterization (RHC) within 5 years prior to Baseline with the following parameters: 1. Pulmonary vascular resistance (PVR) ˃3 Wood Units (WU) (320 dynes.sec.cm-5) 2. A left ventricular end diastolic pressure (LVEDP) or pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg. 3. A mean pulmonary arterial pressure (mPAP) of ≥ 25 mmHg. 4. 6MWD ≥ 100 meters and ≤ 450 meters. 5. WHO Functional Class II-IV. 6. Forced Vital Capacity ≥ 40% predicted within last 6 weeks prior to screening. 7. Females of childbearing potential must have a negative pre-treatment pregnancy test (urine). 8. Age between 18 and 85 years (inclusive) 9. Clinically stable for at least 4 weeks prior to Baseline in the opinion of the Investigator. 10. If on therapy for their parenchymal lung disease and/or sarcoidosis, then the subject should be on a stable well-tolerated dose of the medication(s) for at least 4 weeks prior to enrollment.

Exclusion Criteria:

1. Use of any type of PAH specific therapies. 2. Episodes of disease worsening within 3 months prior to Baseline. 3. Pregnant or breastfeeding females at Screening. 4. Administered L-arginine within 1 month prior to Screening. 5. Any subject who has been enrolled in any previous clinical study with inhaled NO administered through pulsed delivery. 6. On more than 6 L/min of oxygen at rest by nasal cannula for less than 4 weeks. 7. Evidence of any connective tissue disease with FVC > 60% in the last 6 months prior to screening unless there is evidence of moderate to severe fibrosis on CT scan in the opinion of the local radiologist/Investigator. 8. Evidence of clinically significant combined pulmonary fibrosis with emphysema (CPFE) if > 15% of lung fields by CT scan show evidence of emphysema in the opinion of the local radiologist/Investigator. 9. For subjects with sarcoidosis, clinically significant evidence of pulmonary fibrosis on CT scan in the opinion of the local radiologist/Investigator and FVC ≥80% predicted. 10. For subjects continuing on open label therapy, the concurrent use of the INOpulse device with a CPAP/BiPAP, or any other positive pressure device. 11. Significant heart failure in the opinion of the Investigator. 1. LVEF<40% or. 2. PCWP on last RHC>15 mmHg (unless concurrent LVEDP <15 mmHg) or. 3. Significant diastolic dysfunction on echocardiogram

Trial Details

Trial ID:

This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.


Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans.

Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data.

Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs.

Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.

Phase 2
Lead Sponsor

The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.

Principal Investigator

The person who is responsible for the scientific and technical direction of the entire clinical study.

Edward Parsley, DO
Principal Investigator Affiliation Bellerophon Pulse Technologies
Agency Class

Category of organization(s) involved as sponsor (and collaborator) supporting the trial.

Overall Status Recruiting
Countries United States

The disease, disorder, syndrome, illness, or injury that is being studied.

Pulmonary Hypertension, Pulmonary Fibrosis, Sarcoidosis, Pulmonary
Additional Details

A phase 2b, open label study to assess the hemodynamic effects of increasing doses of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary hypertension associated with pulmonary fibrosis or sarcoidosis on long term oxygen therapy followed by an open label extension study

Arms & Interventions


Experimental: PH-Pulmonary Fibrosis

Part 1: 1st 4 subjects will be treated with iNO 30, 45, 75 mcg/kg IBW/hr 2nd 4 subjects will be treated with iNO 45, 75, 125 mcg/kg IBW/hr Part 2: Optional open label long term extension at the optimal dose as identified in Part 1

Experimental: PH-Sarcoidosis

Part 1: 1st 4 subjects will be treated with iNO 30, 45, 75 mcg/kg IBW/hr 2nd 4 subjects will be treated with iNO 45, 75, 125 mcg/kg IBW/hr Part 2: Optional Open label long term extension at the optimal dose as identified in Part 1


Combination Product: - iNO

inhaled nitric oxide

Contact a Trial Team

If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.

University of Miami, Miami, Florida




University of Miami

Miami, Florida, 33125

Site Contact

Emmanuelle Simonet



University of Cincinnati, Cincinnati, Ohio




University of Cincinnati

Cincinnati, Ohio, 45219

Site Contact

Joyce Zigler



Temple University, Philadelphia, Pennsylvania




Temple University

Philadelphia, Pennsylvania, 19140

Site Contact

Dee Fehrlee



Vanderbilt University Medical Center, Nashville, Tennessee




Vanderbilt University Medical Center

Nashville, Tennessee, 37232

Site Contact

Jim DelGreco



Inova Heart and Lung Vascular Institute, Falls Church, Virginia




Inova Heart and Lung Vascular Institute

Falls Church, Virginia, 22042

Site Contact

Serina Zorrilla



University of Washington Medical Center, Seattle, Washington




University of Washington Medical Center

Seattle, Washington, 98195

Site Contact

Chessa Goss



For more information, please contact PHA at Research@PHAssociation.org and refer to the terms of service below.

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