Bosentan in Systemic Sclerosis (HOME)

Inclusion Criteria:

• Male and female subjects > 18 years diagnosed with SSc;
• Reduction of blood flow measured by laser Doppler imaging, of at least 50%, distally to the proximal interphalangeal joint, compared to the healthy volunteers;
• Women of childbearing potential must have a negative pregnancy test and use a reliable form of contraception;
• A history of 1 or more DUs within 2 years prior to inclusion;
• No use of bosentan in the past;
• Subjects willing and able to sign informed consent.

Exclusion Criteria:

• Parenteral prostanoid treatment for DU < 3 months ago;
• Chronic treatment with PDE-5 inhibitor or ERA;
• History of bosentan use
• Irreversible significant limitation of the hand function, e.g. amputation of more than one finger;
• Other types of system- or connective tissue diseases;
• Significant peripheral (macro-) vascular disease due to e.g. diabetes, hyperlipidemia, uncontrolled systemic hypertension, coagulopathy;
• Any serious medical co morbidity (eg, active malignancy) such that the subjects life expectancy is < 12 months;
• Known AST and/or ALT elevations higher than 3 times Upper Limit Normal (ULN);
• Moderate to severe liver function disorder;
• Pregnancy or breastfeeding;
• Treatment with Glibenclamide, Fluconazole, Cyclosporin A, Tacrolimus or other calcineurin inhibitors;
• Hypersensitivity for bosentan or one of its components;
• Subjects not able to follow the protocol.