Systemic sclerosis (scleroderma)

Definition

Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.

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

• Wrist pain
• Wheezing
• Skin, abnormally dark or light
• Joint pain
• Hair loss
• Eye 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.

Treatment

Medicines that treat inflammation and pain include aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), or corticosteroids. Other treatments are directed at specific manifestations of systemic sclerosis: for example, antacids or intestinal motility drugs for heartburn and related symptoms, medications that increase blood flow for Raynaud's phenomenon, and blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems.

Treatment usually includes a combination of physical therapy and skin and joint protection techniques (for example, avoiding cold in the case of Raynaud's phenomenon).

Support Groups

See 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.

Illustrations

Raynaud's phenomenon

CREST syndrome

Sclerodactyly

Telangiectasia

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