Abstract
This review aims to present how a better understanding of the pathogenesis of tissue fibrosis and of the pivotal role of extracellular matrix components supports the use of various immunosuppressive drugs to treat systemic sclerosis (SSc). Recent experimental data demonstrating the antifibrotic effect of several immunosuppressive drugs, independently of their immunosuppressive action, must be put into context with the clinical results observed in immunosuppressed SSc patients. Ciclophosphamide is now considered the drug of choice for interstitial lung disease in SSc and in rapidly progressive diffuse SSc. Since 1996, this drug has been used at higher doses for autologous hematopoietic stem cell transplantations, permitting rapid and durable clinical and histological regression of skin fibrosis.
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