This prospective open-label matched-control pilot study evaluates whether inhaled treprostinil improves exercise pulmonary vascular resistance and functional outcomes in adults with interstitial lung disease or connective tissue disease and exercise-induced pulmonary hypertension. Participants will receive inhaled treprostinil plus standard care or standard care alone and will be followed for 6 months, with an optional extension to 12 months.
Age ≥18 years
ILD diagnosis or CTD diagnosis
WHO Functional Class II-III
mPAP ≤20 mmHg
Resting pulmonary hypertension
Significant left heart disease
CTEPH
Acute PE within 6 months
Parallel
Treprostinil
DrugDescription: * Initial dose 18 mcg four times daily * Titrated to 54-72 mcg four times daily as tolerated Standard disease-specific therapy continues
Standard of Care
Other interventionStandard management of ILD/CTD without treprostinil.
Change in Exercise Pulmonary Vascular Resistance (PVR)
Change in exercise pulmonary vascular resistance (PVR) from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in exercise PVR between the inhaled treprostinil and standard-of-care groups
Incidence of Adverse Events and Treatment Discontinuations
Incidence of adverse events, serious adverse events, treatment discontinuations, and medication tolerability during treatment with inhaled treprostinil.
Progression to Resting Pulmonary Hypertension
Proportion of participants who develop resting pulmonary hypertension at Month 12, defined as resting mean pulmonary artery pressure (mPAP) \>20 mmHg and pulmonary vascular resistance (PVR) \>2 Wood Units measured by right heart catheterization
Change in Exercise Pulmonary Vascular Resistance (PVR) at Month 12
Change in exercise pulmonary vascular resistance (PVR), measured in Wood Units (WU), from baseline to Month 12 among participants continuing in the extension phase.
Change in Right Ventricular Stroke Work Index (RVSWI) at Month 6
Change in right ventricular stroke work index (RVSWI) from baseline to Month 6, measured during exercise right heart catheterization with invasive cardiopulmonary exercise testing (iCPET). The primary analysis will compare the change in RVSWI between the inhaled treprostinil and standard-of-care groups.
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