Treatment of systemic sclerosis

Systemic sclerosis (SSc, scleroderma) is a multisystem disease with a bad prognosis. No drugs have been shown to cure this disease. In view of its pathogenesis, in which immune responses are presumed to play an important role, immune suppressive therapies are widely applied in the treatment of SSc. Recent placebo-controlled trials with cyclophosphamide in patients with SSc complicated by alveolitis, show a modest but significant difference in pulmonary function tests in favour for the cyclophosphamide treated group. Treatment with prostacyclin analogues and endothelin-1 receptor antagonists appear to stabilize pulmonary arterial hypertension, a thus far fatal complication of SSc.