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

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.

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 fibrosisIdiopathic nonspecific interstitial pneumoniaRespiratory bronchiolitis-interstitial lung diseaseDesquamative interstitial pneumoniaCryptogenic organizing pneumoniaAcute interstitial pneumoniaRare IIPs diagnosis by one of the following:Idiopathic lymphoid interstitial pneumoniaIdiopathic pleuroparenchymal fibroelastosisUnclassifiable idiopathic interstitial pneumoniasChronic hypersensitivity pneumonitisOccupational lung diseaseHave been using oxygen therapy by nasal cannula for at least 4 weeks6-Minute Walk Distance (6MWD) ≥ 100 meters and ≤ 450 meters at screening and Baseline/Randomization visitsWorld Health Organization (WHO) Functional Class II-IVForced Vital Capacity ≥ 40% predicated within the last 6 months prior to the screening run-in periodAge between 18 and 85 years (inclusive)Exclusion Criteria:Pregnant or breastfeeding females at ScreeningIn 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