This study aims to evaluate the efficacy and safety of inhaled treprostinil in subjects with sarcoidosis-associated interstitial lung disease and pulmonary hypertension.
Pulmonary sarcoidosis-associated pulmonary hypertension is classified as WHO Group 5 pulmonary hypertension and may occur in anywhere from 5-20% of sarcoidosis patients. Inhaled treprostinil has shown clinical improvements in exercise capacity after 12 weeks of therapy in patients with WHO Group 1 pulmonary hypertension. More recently, there has been interest in using inhaled PAH-specific therapies for the treatment of pulmonary hypertension associated with interstitial lung disease. The investigators believe that those patients with pulmonary hypertension in the setting of sarcoidosis-associated interstitial lung disease are a unique population which may potentially benefit from inhaled, targeted pulmonary arterial hypertension therapy (inhaled treprostinil) while minimizing the adverse effects associated with systemic pulmonary vasodilators. This study aims to evaluate the efficacy and safety of inhaled treprostinil in subjects with sarcoidosis-associated interstitial lung disease and pulmonary hypertension.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
5
Inhaled treprostinil causes dilatation of the pulmonary arteries and may help reduce the pulmonary pressures in this studied population. All subjects will initiate inhaled treprostinil at a dose of 3 breaths (18 mcg) four times daily. Study drug doses escalations (additional one breath four times daily) can occur every three days with a maximum dosing regimen of up to 12 breaths (72 mcg) four times daily, as clinically tolerated.
University of Florida, Division of Pulmonary and Critical Care Medicine
Gainesville, Florida, United States
PVR by Right heart catheterization (RHC)
Change in RHC parameter PVR (pulmonary vascular resistance )
Time frame: Baseline, Week 16
mPAP by Right heart catheterization (RHC)
Change in RHC parameter mPAP (mean pulmonary arterial pressure)
Time frame: Baseline, Week 16
Change in 6-Minute Walk Test (6MWT)
change in walk test distance during the study
Time frame: Baseline, Week 8, Week 16
Change in Cardiac MRI parameters
Change in Right ventricle ejection fraction, Right ventricular end diastolic ventricle index, right ventricular systolic index
Time frame: Baseline, Week 16
Change in Pulmonary Function Testing
Change in FEV1 abd FVC
Time frame: Baseline, Week 16
Change in Brain Natriuretic Peptide (BNP)
change in BNP level during the study
Time frame: Baseline, Week 16
Change in WHO Functional Class (WHO FC)
change in WHO FC status during the study
Time frame: Baseline, Week 8, Week 16
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