Abstract

The uniform failure of trials using potent nonselective immunosuppressive and cytotoxic agents to halt the progression of SSc indicate an urgent need for alternative types of therapeutics in future trials. The improved outcome among SSc patients in the past 20 years primarily has been secondary to the widespread use of ACE inhibitors, calcium channel blockers, and improved pharmacologic agents to manage gastrointestinal symptoms. It remains unknown whether early treatment with D-penicillamine or aggressive treatment of interstitial lung disease will slow progression of cutaneous or pulmonary fibrosis, respectively. Recent advances and accelerated research into the mechanisms of cellular activation, growth, and regulation hold great potential for understanding the pathogenesis of SSc. Hopefully the next decade will yield new information regarding fibroblast biology, endothelial cell injury, and human immune regulation so that specific biologic response agents may selectively halt disease progression. Progress probably will result only from well-designed multicenter trials of patients in the early stages of illness involving quantifiable outcome measures of disease.

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