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Systemic sclerosis (SSc, scleroderma) is a disease characterized by an increase in synthesis and deposition of extracellular matrix, resulting in fibrosis and microvascular injury of the skin and internal organs. Two subtypes which define the degree of disease involvement are recognized: diffuse cutaneous and limited cutaneous. The disease spares children and increases in prevalence with age, occurring most frequently in women of childbearing age, and most frequently and severely in black women. Cardiac,4 pulmonary5 and renal6 involvement are among the most severe complications of the diffuse form of the disease. Individuals with scleroderma have a significant decrease in lifespan, with a ten-year survival from diagnosis of <50%.7 Pharmacologic treatment of scleroderma includes 5-fluorouracU, D-penicillarnine, dipyrida- mole, and para-aminobenzoic acid,8 in addition to pharmaceuticals aimed at controlling the symptoms of organ involvement. Disease progression leads to a decrease in dermal pliability with a resultant loss of range of motion in the joints. Progressive fibrosis results in a progressive reduction in pulmonary function and failure of the internal organs occurs with time. The pathogenesis of scleroderma is poorly understood; however, a disruption in the immune system, the fibroblast cells and the vasculature have all been identified. |
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