A phase 2, open-label, multi-center clinical study to evaluate the safety and efficacy of inhaled mosliciguat in participants with pulmonary hypertension associated with interstitial lung disease (PH-ILD) on a background inhaled treprostinil

Inclusion criteria

• Age 18-85 years old
• Diagnosis of Interstitial Lung Disease (confirmed by central read of HRCT scan) showing diffuse parenchymal disease
  • Eligible ILD subgroups: IIP, chronic hypersensitivity pneumononitis, CPFE, ILD associated with CTD
    • FVC<70% predicted for CTD
  • Evidence of fibrotic component and limited emphysema component
• Confirmed pulmonary hypertension (PH)  by right heart catheterization (RHC) during screening with evidence of elevated pulmonary vascular resistance (PVR)
• Receiving a stable dose of inhaled treprostinil by dry powder inhaler, stable for >3 months at the time of screening.
• Able to walk 100 meters in 6 minutes

Exclusion Criteria

• Diagnosis of PH Group 1, 2, 4, or 5.
• Diagnosis of PH Group 3 outside of disease subgroups listed in inclusion criterion
• Currently on PH therapy other than DPI treprostinil, including but not limited to: PDE5 inhibitors (eg, sildenafil), prostacyclin receptor agonists (eg, selexipag), endothelin receptor antagonists (eg, ambrisentan), or activin inhibitors (eg, sotatercept)
• History of intolerance to or lack of efficacy with mosliciguat or sGC stimulators or activators
• Evidence of clinically significant left-sided heart disease: PCWP >15 mmHg or LVEF <40%