Clinical Trial Finder

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

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

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

Interventions

Combination Product: - iNO

inhaled nitric oxide