Systemic Sclerosis (Scleroderma) |
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Alternate Names: CREST Syndrome, Progressive Systemic Sclerosis, Scleroderma
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Definition |
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Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.
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Introduction
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Scleroderma is a disease of the skin, joints and sometimes, internal organs. Its cause is not known. Scleroderma means hardening (fibrosis) of the skin, and due to malfunction of the vascular and immune systems, results in the overproduction of collagen (connective tissue in the body). Scleroderma can be localized to the skin or may become systemic with excess collagen building up in various organs such as the esophagus, kidneys, lungs, gastrointestinal tract, heart skin, and peripheral nervous system.
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Fortunately, scleroderma is relatively rare affecting approximately 300,000 persons in the United States. The disorder most commonly occurs in women between the ages of 20 and 40, however men and children can be affected as well. The disease is not contagious and is not thought to be inherited.
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Overview, Causes, & Risk Factors
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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.
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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.
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Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract.
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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.
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Symptoms & Signs
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• blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
• pain, stiffness, and swelling of fingers and joints
• skin thickening and shiny hands and forearm
• skin is hard
• tight and mask-like facial skin
• ulcerations on fingertips or toes
• esophageal reflux or heartburn
• difficulty swallowing
• bloating after meals
• weight loss
• diarrhea
• constipation
• shortness of breath
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Additional symptoms that may be associated with this disease:
• wrist pain
• wheezing
• skin, abnormally dark or light
• joint pain
• hair loss
• eye burning, itching and discharge
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Currently, there is no know cure for scleroderma. Early diagnosis is important to enable initiation of treatment to prevent long-term complications. The primary goals of treatment are to relieve and control symptoms, improve quality of life, and prevent long-term complications.
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Treatment options for sclerderma vary depending upon whether the disease is localized or systemic and the extent of the damage to various organ systems. In general, medications that may be used include immunosuppressive agents, antifibrotic agents, anti-inflammatory agents, vasodilators, and angiotensin-converting enzyme inhibitors. In rare cases, when symptoms of systemic sclerosis become severe, surgery may be recommended.
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