ZEBRA Clinical Trial Testing Treatment for Scleroderma Renal Crisis

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by Maureen Newman |

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kidney disease due to scleroderma

SSc clinical trialZibotentan Better Renal Scleroderma Outcome Study (ZEBRA)” is more than a standard clinical trial for scleroderma patients. Rather, the phase 2 clinical trial is a three-part clinical trial seeking to help patients with mild, moderate, or severe kidney disease due to scleroderma.

University College, London is sponsoring the trial in collaboration with the Medical Research Council. The aim is to study Zibotentan in patients with mild or moderate kidney disease in ZEBRA 1, severe acute kidney disease (renal crisis) without dialysis in ZEBRA 2A, and renal crisis with dialysis in ZEBRA 2B.

Zibotentan is thought to alleviate the symptoms of kidney disease in patients with scleroderma because it is a selective endothelin blocker. According to “Endothelin-1 Expression in Scleroderma Renal Crisis,” an article published in Human Pathology, endothelin-1 is over-expressed in the kidneys of patients with scleroderma, suggesting the protein’s involvement in the disease.

During the trial, patients will be monitored for soluble Vascular Cell Adhesion Molecule (sVCAM1) because it is a downstream result of endothelin-1. “Endothelin-1 stimulates small artery VCAM-1 expression through p38MAPK-dependent neutral sphingomyelinase,” according to an article published in Journal of Vascular Research. By blocking endothelin-1, Zibotentan may bring relief to the patients studied in this trial.

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Kidney disease is common in those with the systemic forms of scleroderma, and patients with SSc are typically screened for kidney disease regularly. According to Sclero.org, about 20% of people with diffuse scleroderma as well as limited scleroderma and CREST Syndrome develop high blood pressure and kidney failure, also known as scleroderma renal crisis. As a result, ZEBRA’s results could help fulfill a major medical need for those with systemic forms of Scleroderma who are at risk for severe kidney failure.