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.

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 test

febrile/cold agglutinins

esophageal manometry

ACE 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 failure

renal failure

pulmonary fibrosis

malabsorption

(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.