SYSTEMIC-SCLEROSIS - CURRENT PATHOGENETIC CONCEPTS AND FUTURE-PROSPECTS FOR TARGETED THERAPY

Although there is no cure for systemic sclerosis, the disease is certa inly treatable. And the more that is understood of its pathogenesis, t he better the prospects for treatment. Excessive collagen deposition i n the skin and viscera is responsible for the morbidity and mortality. The immune system plays a critical role in setting up the fibrotic pr ocess, but impaired regulation of collagen gene expression probably un derlies the persistence of fibrosis. The development of effective anti fibrotic drugs, in particular agents that inhibit transcription factor s involved in the activation of type I collagen genes, is likely to be the way forward for treatment of the later stages of diffuse systemic sclerosis, with immunomodulatory therapy for the earlier stages.