Many patients with scleroderma have damage to their kidneys caused by the disease. There is limited evidence for treatments to prevent this damage or stop it progressing. Blocking a substance in the blood called endothelin has helped treat some aspects of scleroderma. The purpose of this study is to see how effective a new endothelin blocker called Zibotentan is in treating patients who have scleroderma and have gone on to develop reduced kidney function as a complication. It will be given in addition to the accepted treatments used for scleroderma. There will be three parts to this study each for a different group of patients:
* ZEBRA 1 for patients with mild or moderate kidney disease caused by scleroderma
* ZEBRA 2A for patients with a more severe, acute form of kidney disease caused by scleroderma (scleroderma renal crisis) who do not require dialysis
* ZEBRA 2B for patients who have had scleroderma renal crisis and are on dialysis
Who can participate
Age range18 Years
SexALL
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Inclusion criteria
✓. Adults with scleroderma and:
✓. CKD 2/3 (ZEBRA 1)
✓. Renal crisis not on dialysis (ZEBRA 2A)
✓. Renal crisis on dialysis (ZEBRA 2B)
Exclusion criteria
✕. Previous use of an endothelin receptor antagonist within 3 months of the study start
✕. Significant abnormalities in liver function testing (ALT, ALP, Bilirubin) more than three times upper limit of normal)
✕. Patients with body weight \<40kg.
✕. Patients with conditions which prevent compliance with the protocol or failure to adhere to therapy.
✕. Patients with any other life threatening condition.
✕. Patients with known hypersensitivity to Zibotentan or its excipients
. Previous history of epilepsy or other CNS AEs, neurologic symptoms or signs consistent with acute or evolving spinal cord compression, and CNS metastases
✕. Patients with a baseline left ventricular ejection fraction \< 40% (prior to any scleroderma renal crisis), patients with acute myocardial infarction within six months or patients who are judged by the trial clinician to be at unacceptable risk from cardiac complications.