Systemic sclerosis

Systemic sclerosis is a disease characterized by several distinctive features. This disease spares children, and its incidence increases steadily with age. Women are affected more than men, especially during childbearing years. Although there is no overall racial predilection, SSc occurs most frequently and severely in young black women. Family and genetic studies suggest only a weak genetic predisposition, but there are many occupational agents that may be implicated in the pathogenesis. The disease with its involvement in many organ systems results in a significant reduction in lifespan. Improvement in survival of renal crisis has been dramatic with the use of ACE inhibitors. Further epidemiologic studies using subsets of patients with more homogeneous clinical and laboratory features will lead to a better understanding of this complex disease.