A Study in Participants With Sarcoidosis-associated Pulmonary Hypertension (SAPH) to Assess the Efficacy and Safety of Oral Selexipag (SPHINX)
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ClinicalTrials.gov Identifier: NCT03942211 |
Recruitment Status :
Active, not recruiting
First Posted : May 8, 2019
Last Update Posted : February 1, 2023
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Condition or disease | Intervention/treatment | Phase |
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Sarcoidosis-associated Pulmonary Hypertension | Drug: Selexipag Drug: Placebo | Phase 2 |
Study Type : | Interventional (Clinical Trial) |
Actual Enrollment : | 10 participants |
Allocation: | Randomized |
Intervention Model: | Parallel Assignment |
Masking: | Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor) |
Primary Purpose: | Treatment |
Official Title: | A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study in Participants With Sarcoidosis-Associated Pulmonary Hypertension (SAPH) to Assess the Efficacy and Safety of Oral Selexipag. |
Actual Study Start Date : | February 26, 2021 |
Estimated Primary Completion Date : | April 16, 2023 |
Estimated Study Completion Date : | September 15, 2024 |

Arm | Intervention/treatment |
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Experimental: Selexipag 200 micro gram (μg)
Study intervention will be up-titrated to allow each participant to reach their individual maximum tolerated dose (iMTD), in the range of 200 μg to1600 μg (ie, 1 to 8 tablets) twice daily/once daily. Dosing frequency will be twice daily, except for participants with moderate hepatic impairment (Child-Pugh Class B) or who are concomitantly taking (a) moderate CYP2C8 inhibitor(s), who receive study intervention once daily. The dose will be up-titrated by the investigator/delegate in 200 μg twice daily/once daily increments at weekly intervals during scheduled TCs until reaching the iMTD. If the dose regimen is not well tolerated or symptoms cannot be fully managed with symptomatic treatment, the duration of the titration step can be prolonged to 2 weeks. If needed, the dose can be reduced by 200 μg twice daily/once daily.
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Drug: Selexipag
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
Other Name: JNJ-678896049; ACT-293987 |
Placebo Comparator: Placebo
The comparator will be administered similarly to the experimental intervention.
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Drug: Placebo
Oral tablets without active compound. Participants can receive 1 to 8 tablets at each administration. |
- Pulmonary Vascular Resistance (PVR) on Study Intervention up to Week 26 [ Time Frame: Up to week 26, within 2-5 hours post-dose ]PVR is measured by right heart catheterization (RHC) and expressed as percent of baseline value.

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Ages Eligible for Study: | 18 Years to 75 Years (Adult, Older Adult) |
Sexes Eligible for Study: | All |
Accepts Healthy Volunteers: | No |
Main Inclusion Criteria:
- Confirmed diagnosis of sarcoidosis as per American Thoracic Society (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 treatment with PH-specific treatment or oral PH-specific monotherapy (ie, riociguat or PDE5i or ERA); if on oral PH-specific monotherapy then treatment had to be stable (ie, no introduction of new therapies or changes in dose) for at least 90 days prior to both and 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
- 6-minute walk distance (6MWD) greater than or equal to (>=) 50 meters both at Screening and at the time of randomization. Participants can use their usual walking aids during the test (example, cane, crutches). The same walking aid should be used for all 6-minute walk test (6MWTs). Walkers are not allowed
- Forced Vital Capacity (FVC) greater than (>) 50 percent (%) and Forced Expiratory Volume (in 1 second) (FEV1) > 50% of predicted at Screening
- Diffusing capacity of the lung for carbon monoxide (DLCO) >= 40% of predicted. If DLCO less than (<) 40% of predicted, the extent of emphysema should not be greater than that of fibrosis as assessed by high resolution computerized tomography (CT) scan
- 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 agreeing to remain on an acceptable method while receiving study intervention and until 30 days after last dose of study intervention
- A woman only using hormonal contraceptives must have been using this method for at least 30 days prior to randomization
Main 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) <40%.
- Treatment with prostacyclin, prostacyclin analogues or IP receptor agonists (ie, selexipag) within 90 days prior to randomization and/or prior to the RHC qualifying for enrollment, except those given at vasodilator testing during RHC.
- SBP <90 mmHg at Screening or at randomization.
- Included on a lung transplant list or planned to be included until Visit 6 / Week 39.
- Change in dose or initiation of new diuretics and/or calcium channel blockers within 1 week prior to RHC qualifying for enrollment.
- Any condition for which, in the opinion of the investigator, participation would not be in the best interests of the participant (eg, compromise well-being), or that could prevent, limit, or confound the protocol-specified assessments.
- Any acute or chronic impairment that may influence the ability to comply with study requirements such as to perform RHC, a reliable and reproducible 6MWT, or lung function tests.
- Any other criteria as per selexipag Summary of Product Characteristics (SmPC)

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03942211

Study Director: | Rainer Zimmermann | Actelion |
Responsible Party: | Actelion |
ClinicalTrials.gov Identifier: | NCT03942211 |
Other Study ID Numbers: |
AC-065D301 2018-004887-74 ( EudraCT Number ) |
First Posted: | May 8, 2019 Key Record Dates |
Last Update Posted: | February 1, 2023 |
Last Verified: | January 2023 |
Individual Participant Data (IPD) Sharing Statement: | |
Plan to Share IPD: | Yes |
Plan Description: | Actelion is a Janssen pharmaceutical company of Johnson & Johnson. The data sharing policy of the Janssen Pharmaceutical Companies of Johnson & Johnson is available at www.janssen.com/clinical-trials\transparency. As noted on this site, requests for access to the study data can be submitted through Yale open Access (YODA) Project site at yoda.yale.edu |
URL: | https://www.janssen.com/clinical-trials/transparency |
Studies a U.S. FDA-regulated Drug Product: | Yes |
Studies a U.S. FDA-regulated Device Product: | No |
selexipag, sarcoidosis, pulmonary hypertension |
Hypertension, Pulmonary Hypertension Lung Diseases Selexipag Sarcoidosis Vascular Diseases |
Cardiovascular Diseases Respiratory Tract Diseases Lymphoproliferative Disorders Lymphatic Diseases Antihypertensive Agents |