| Subject | Contents |
| Definition | A diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. |
| Alternative Names | CREST syndrome; Progressive systemic sclerosis; Scleroderma |
| 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. |
| Symptoms | 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 heartburndifficulty swallowingbloating after meals weight lossdiarrheaconstipationshortness of breath Additional symptoms that may be associated with this disease: wrist painwheezingskin, abnormally dark or lightjoint painhair losseye burning, itching and discharge |
| Signs and tests | Examination of the skin may show tightness, thickening, and hardening. The ESR is elevated. The rheumatoid factor can be elevated. An antinuclear antibody test is usually positive. Urinalysis can show protein and microscopic blood. A chest X-ray may show fibrosis. Pulmonary function studies often show restrictive lung disease . A skin biopsy may also be performed. This disease may also alter the results of the following tests: LE cell testfebrile/cold agglutininsesophageal manometryACE levels |
| Treatment | See scleroderma treatment. |
| Support Groups | The stress of illness can often be helped by joining a support group where members share common experiences and problems. See systemic sclerosis (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. |
| Complications | cardiac failurerenal failurepulmonary fibrosismalabsorption (inadequate absorption of nutrients from the intestinal tract) |
| Calling your health care provider | Call 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. |
| Prevention | There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride. |
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