CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Mixed connective disease; Morphea - linear
Scleroderma is a connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. It is a type of autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue.
The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.
The disease usually affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
Some types of scleroderma affect only the skin, while others affect the whole body.
Skin symptoms of scleroderma may include:
Bone and muscle symptoms may include:
Breathing problems may result from scarring in the lungs and can include:
Digestive tract problems may include:
The health care provider will perform a physical exam. The exam may show hard, tight, thick skin.
Your blood pressure will be checked. Scleroderma can cause severe inflammation of small blood vessels, such as those in the kidneys. Problems with your kidneys can lead to high blood pressure.
Blood tests may include:
Other tests may include:
There is no specific treatment for scleroderma.
Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:
Other treatments for specific symptoms may include:
Treatment usually also involves physical therapy.
Some people with scleroderma have symptoms that develop quickly over the first few years and continue to get worse. However, in most patients, the disease slowly gets worse.
People who only have skin symptoms have a better outlook. Widespread (systemic) scleroderma can damage the heart, kidney, lungs, or GI tract, which may cause death.
Lung problems are the most common cause of death in patients with scleroderma.
The most common cause of death in people with scleroderma is scarring of the lungs, called pulmonary fibrosis.
Other complications of scleroderma include:
Call for an appointment with your health care provider if:
There is no known prevention. Reducing your exposure to silica dust and polyvinyl chloride may lower your risk for this disease.
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Varga J, Denton CP. Systemic sclerosis and the scleroderma-spectrum disorders. In: Firestein GS, Budd RC, Harris ED Jr., et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008:chap 77.
Clouse RE, Diamant NE. Esophageal motor and sensory function and motor disorders of the esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006:chap 41.
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