Systemic Sclerosis (Scleroderma) Clinical Trial
Systemic sclerosis (ssc) is characterised by extensive tissue fibrosis. Using drugs that are capable of inhibiting fibroblast activity may be beneficial if administrered early in the disease course. Thirty adult patients with early SSc will be treated with the endothelin-1 antagonist bosentan for 6 months.Disease progression will be assessed.
Systemic sclerosis (SSc) is characterised by obliterative vasculopathy and extensive
fibrosis. The accumulation of extracellular components in the extracellular matrix is mostly
due to increased activity og tissue fibroblasts. The proliferation and hyperactivity of the
fibroblasts may be caused by enhanced production of several cytokins, among them
endothelin-1.The activity of endothelin-1 has been shown to be increased both in the
circulation and within skin lesions. Endothelin-1 has several distinct properties, among
them profibrotic activity, inflammatory and vasoconstriction.Thus, the actions induced by
endothelin-1 may be a potensial target for the therapy of SSc.
Thirty patients with early SSc, that is of less than 12 months duration will be offered six
months of treatment with the oral dual endothelin-1 antagonist bosentan. Assessment of
disease progression will be performed at 3, 6, 9. 12 and 24 months using clinical,
histological and immunohistochemical methods.
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Allocation: Non-Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment