Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by excessive collagen deposition, vascular damage, inflammation, and progressive fibrosis of the skin and visceral organs. Race has a significant influence on the epidemiology, clinical manifestations, survival, autoantibody frequencies, and genetic factors in SSc.
Multiple studies in African-Americans and Whites have implicated the interaction between racial background, autoantibody subtype, and genetic factors in determining disease manifestations, severity, and progression in SSc. African-Americans have been reported to have a more severe clinical phenotype, with younger age at SSc onset, higher frequency of diffuse skin involvement, more extensive pulmonary disease, higher risk for scleroderma renal crisis, and an overall worse prognosis (including higher mortality) compared to Whites even after controlling for socioeconomic factors.
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