High blood pressure in lung vessels associated with sarcoidosis Sarcoidosis associated pulmonary hypertension
Conditions
Interventions
Selexipag 200 micro gram (µg): Oral tablets containing 200 µg of selexipag.
Depending on the iMTD, participants will receive 1 (200 µg) to 8 (1600 µg)
tablets at each administration.
Study intervent
Sponsors
Janssen-Cilag
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: - Male or female -18 to 75 years of age, inclusive - Confirmed diagnosis of sarcoidosis as per ATS criteria - Sarcoidosis-associated precapillary PH, confirmed by RHC (at rest) within 90 days prior to randomization. - PH severity according to modified WHO FC II-IV at Screening and randomization; participants in WHO FC IV must be in a stable condition and able to perform a 6MWT. - Either not receiving PH-specific treatment, or receiving PH-specific oral monotherapy (ie, riociguat or PDE5i or ERA); if on oral PH-specific monotherapy treatment has to be stable (ie, no introduction of new therapies or changes in dose) for at least 90 days prior to both the RHC qualifying for enrollment and randomization. - Stable sarcoidosis treatment regimen, ie, no new specific anti inflammatory treatment for sarcoidosis for at least 90 days, and stable dose(s) for at least 30 days prior to both the RHC qualifying for enrollment and randomization. - 6MWD between 50 and 450 m both at Screening and at the time of randomization. - Forced vital capacity (FVC) >50% of predicted at Screening. - FEV1/FVC >=60%, or if FEV1/FVC =60% of predicted at Screening. - Women of childbearing potential must have a negative pregnancy test at screening and randomization, must agree to undertake monthly urine pregnancy tests, and to practice an acceptable method of contraception and agree to remain on an acceptable method while receiving study intervention and until 30 days after last dose of study intervention. - A woman using oral contraceptives must have been using this method for at least 1 month prior to randomization.
Exclusion criteria
Exclusion criteria: - PH due to left heart disease (PAWP >15 mmHg). - History of left heart failure (LHF) as assessed by the investigator including cardiomyopathies and cardiac sarcoidosis, with a left ventricular ejection fraction (LVEF)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Pulmonary Vascular Resistance (PVR) on Study Intervention up to Week 26, 2-5h post dose. PVR is measured by right heart catheterization (RHC) and expressed as percent of baseline value. | — |
Secondary
| Measure | Time frame |
|---|---|
| No secondary outcome measures, rest of outcomes is exploratory. | — |
Countries
Netherlands
Outcome results
None listed