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Systemic sclerosis (scleroderma)
Alternative namesCREST syndrome; Progressive systemic sclerosis; Scleroderma
DefinitionScleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.
Causes, incidence, and risk factors
The cause of scleroderma is unknown. The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected.
Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs also occurs. In the skin, ulceration, calcification, and changes in pigmentation may occur.
Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract.
The disease usually affects people 30 to 50 years old. Women are affected more often than men. Risk factors are occupational exposure to silica dust and polyvinyl chloride.
Signs and testsExamination of the skin may show tightness, thickening, and hardening.
TreatmentSee scleroderma treatment.
Support GroupsSee scleroderma - support group .
Expectations (prognosis)In the majority of those affected, the disease is progressive. In some, remission occurs with a slow progression. People who only have skin involvement have a better prognosis (probable outcome). Death may occur from gastrointestinal, cardiac, kidney, or pulmonary (lung) involvement.
Calling your health care providerCall for an appointment with your health care provider if symptoms of this disorder are present, or if you have scleroderma and symptoms become worse, or if new symptoms develop.
PreventionThere is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.
Update Date: 8/6/2003Megan E. B. Clowse, M.D., M.P.H., Division of Rheumatology, Johns Hopkins Hospital, Baltimore, MD. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT