A Study of Mosliciguat in Combination With Inhaled Treprostinil in PH-ILD

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.

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:

• Idiopathic interstitial pneumonia (IIP)
• Chronic hypersensitivity pneumonitis
• 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.