scleroderma Systemic sclerosis
Conditions
Interventions
Group 1: Usual care AND bosentan 62.5 mg twice daily, titrated to 125 mg twice
daily after one month if tolerated (n=20)
Group 2: Usual care only (n=20)
Group 3: healthy controls, no care (n=20)
Sponsors
Universitair Medisch Centrum Groningen
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: SSc patients * 18 years or older * Systemic sclerosis based on the 2013 ACR/EULAR criteria * Raynaud*s phenomenon (RP) * A history of digital ulcer disease * Assessable Pulse Wave Velocity (PWV) measurement at baseline * Written informed consent;Healthy controls: * 18 years or older * Written informed consent
Exclusion criteria
Exclusion criteria: SSc patients: * Hypersensitivity to the active substance or to any of the excipients * Moderate to severe hepatic impairment, i.e., Child-Pugh class B or C * Baseline values of liver aminotransferases, i.e., aspartate aminotransferases (AST) and/or alanine aminotransferases (ALT), greater than 3 times the upper limit of normal * Concomitant use of cyclosporine A * Pregnancy * Women of child-bearing potential who are not using reliable methods of contraception * Significant peripheral vascular disease as the sole consequence of atherosclerotic disease due to for example diabetes, dyslipidemia, systemic hypertension, coagulopathy;Healthy controls: * Comorbidities
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Mean of right and left carotid-femoral arterial (i.e. aortic) Pulse Wave Velocity (cfPWV) | — |
Secondary
| Measure | Time frame |
|---|---|
| * Right and left carotid-brachial and carotid-radial arterial PWV (cbPWV and crPWV) * Local PWV of the right and left radial (rPWV), brachial (bPWV), femoral (fPWV), and common carotid artery (cPWV) * Mean intima media thickness (IMT) of right and left radial, brachial, femoral, and common carotid artery * Nail-fold Capillary Microscopy and derived indices: Microangiopathy Evolution Score (MES), Capillaroscopic Skin Ulcer Risk Index (CSURI) and Prognostic Index for Digital Laesions (PILD) * Blood flow in the hands, and in different regions of the hands (region of interest (ROI) 1, ROI 2 and ROI 3), as measured by Laser Doppler Perfusion Imaging (LDPI) at standardised ambient hand temperature. * Skin Autofluorescence * Mean number of new DUs per and time to healing of the cardinal ulcer * Urine albumin/creatinine ratio (ACR) * Plasma NT-proBNP and uric acid * Serum levels of matrix metalloproteinases (MMP 3 and MMP 9), and tissue inhibitors of metalloproteinases (TIMP) * Systolic, diastolic, and mean arterial blood pressure of the brachial artery * Modified Rodnan Skin Score (mRSS) * Scleroderma Health Assessment Questionnaire (SHAQ), EuroQol EQ-5D, and SF-36 | — |
Countries
Netherlands
Outcome results
None listed