Description
Inclusion Criteria:
- * Study participant willing and able to provide informed consent
- * Negative urine pregnancy test at baseline for females of childbearing potential
- * Established diagnosis of sarcoidosis by ATS/ERS/WASOG 1999 Statement on of Sarcoidosis
- * Presence of interstitial lung disease by Scadding Stage IV chest radiograph or extensive fibrosis on chest computed tomography
- * Right heart catheterization within six months of baseline visit showing precapillary pulmonary hypertension (mPAP ≥ 25 mmHg, PCWP ≤ 15 mmHg, and PVR \> 3 WU)
- * Patient on stable sarcoidosis therapy for at least three months prior to screening
- * If patients are on oral PAH therapy (PDE5i/SCGS and/or ERA) then dose should be stable for at least three months prior to screening
- * A 6MWT within three months of screening visit of \> 100 meters
Exclusion Criteria:
- * Pregnant patients or those who are actively lactating
- * Patient not willing to use form of birth control (if applicable) during the study
- * Inability to undergo 6MWT, RHC, PFTs or CMRI
- * Predicted survival \< 6 months
- * Patient on any prostanoid or prostanoid analog therapy
- * Patients with left sided heart disease as defined by either a PCWP \> 15 mmHg and/or left ventricular ejection fraction \< 40%
- * Use of any investigational drug/device, or participation in any investigational study with therapeutic intent within 30 days prior to randomization.
Ages Eligible for Study:
18 Years to 99 Years (ADULT, OLDER_ADULT)
Sexes Eligible for Study: ALL
Accepts Healthy Volunteers:
No