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Scleroderma Systemic Sclerosis

What is it?

Scleroderma is an uncommon chronic multisystem disease characterized by a proliferative, destructive abnormality of small- and medium-size blood vessels, excessive collagen deposits in tissues, and an overstimulated immune system. The disease, which means hardening (sclero) of the skin (derma), was initially thought to affect only the skin. It was later recognized that vital organs may be affected as well. There appears to be an early inflammatory component. This may lead fibroblasts and other cells that manufacture the protein collagen (a major component of connective tissue) to overproduce dense, inelastic, scarlike connective tissue. Circulation may be affected as the buildup of dense tissue chokes off blood flow through the tiny capillary blood vessels and small arterioles.When an internal organ (typically the esophagus, lungs, kidneys, or heart) is involved, fibrous tissue slowly infiltrates organ tissues. This may lead to life-threatening conditions such as severe lung disease or kidney failure. Scleroderma usually evolves gradually over a number of years; outcome depends on the organ system involved.

What Causes It?

  • The cause of scleroderma is unknown, although it appears to be an autoimmune disorder (an attack by the body's defenses on healthy tissue). Exposure to silica dust, vinyl chloride, epoxy resins, and hydrocarbons can cause tight, thickened skin over the fingers (sclerodactyly), which differs from scleroderma.

Prevention

  • There is no known way to prevent scleroderma.

Diagnosis

  • Frequently, the diagnosis may be determined upon patient history and physical examination alone.
  • Blood tests and skin biopsy may be taken to rule out other connective tissue disorders.

How to Treat It

  • Treatment is aimed at minimizing symptoms and complications.Occasionally, the disease spontaneously improves.
  • Immunosuppressive drugs such as methotrexate or penicillamine may help in some patients, but there is no solid proof that they improve the outcome.
  • Skin lotions and bath oils may be used to soften the skin. Regular exercise and massage may help to maintain skin pliability as well.
  • Antacids, frequent small meals, and elevation of the head of the bed are recommended for patients with heartburn.Medications such as histamine (H2) blockers or omeprazole may be helpful for esophagitis (inflammation or irritation of the esophagus).
  • Drugs may be prescribed to aid circulation and so reduce the symptoms of Raynaud's phenomenon.
  • Patients with scleroderma may have muscle weakness caused by an associated polymyositis (muscle inflammation, sometimes involving the heart; see also Dermatomyositis). This should be detected early and treated aggressively with corticosteroids.
  • Dialysis may be needed for impaired kidney function; high blood pressure (which occurs when the kidneys are affected) is treated with drug therapy.
  • Patients with heart failure require treatment with digitalis and diuretics.

When to Call a Doctor

  • Call a doctor if patches of skin become tight, shiny, and inelastic or if you experience poor circulation, swallowing difficulty, or shortness of breath.