Systemic sclerosis (scleroderma)

Systemic sclerosis (scleroderma)

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 heartburn
difficulty swallowing
bloating after meals
weight loss
diarrhea
constipation
shortness of breath

Additional symptoms that may be associated with this disease:

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:

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.

Review Date: 11/26/2001
Reviewed By: Birgit Kovacs, M.D., Division of Rheumatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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