Summary
This is a Phase 2, open-label, multi-center clinical study to evaluate the safety and tolerability of inhaled mosliciguat in participants with pulmonary hypertension associated with interstitial lung disease (PH-ILD) on a background inhaled treprostinil.
Detailed Description
This study is an open-label study with an extension.
The study consists of 2 periods: an open label treatment period (16 weeks) and an extension (beyond 16 weeks).
Participants will receive mosliciguat in the 16-week treatment period.
All participants who complete the 16-week treatment period may continue to participate in the extension period where all participants will receive mosliciguat.
Eligibility Criteria
Age Range: 18 years to 85 years
Inclusion Criteria:
* Participants willing and able to provide informed consent
* Participants on inhaled treprostinil
* Participants with diagnosis of Interstitial Lung Disease (ILD). Diagnosis will be confirmed by a high-resolution computerized tomography (HR-CT) scan showing diffuse parenchymal disease. Eligible diagnosed diseases include:
1. Idiopathic interstitial pneumonia (IIP)
2. Chronic hypersensitivity pneumonitis
3. ILD associated with connective tissue disease (CTD) with a forced vital capacity (FVC) \< 70% of predicted
* Confirmed pulmonary hypertension (PH) by right heart catheterization (RHC).
* Ability to perform 6MWD ≥100 meters.
Exclusion Criteria:
* Diagnosis of PH Group 1 (eg. pulmonary arterial hypertension), Group 2 (related to left-heart dysfunction), Group 4 (eg, chronic thromboembolic pulmonary hypertension), or Group 5 (eg, unclassified).
* Exacerbation of underlying lung disease within 28 days prior to randomization.
* Initiation of pulmonary rehabilitation within 28 days prior to randomization.
* Receiving \>10 L/min of oxygen supplementation by any mode of delivery at rest at Baseline.
* History or intolerance to or lack of efficacy with mosliciguat or sGC stimulators or activators.
* Receipt of investigational, or experimental therapy within 42 days OR 5 half-lives prior to randomization.
Note: Other inclusion and exclusion criteria may apply.