Scleroderma

Description

A rare, progressive disease that leads to hardening and tightening of the skin and connective tissues: the fibers that provide the framework and support for your body. Scleroderma can affect only the skin or involve other parts of the body. This disorder is felt to be caused by an over active immune system which attacks the body (auto immune disease). Scleroderma usually starts with a few dry patches of skin on the hands or face that begin getting thicker and harder. These patches then spread to other areas of the skin. The disease can be very serious leading to fibrous scarring of the esophagus, heart, lungs and kidneys, and carries an average 12 year survival rate from the time of diagnosis. Scleroderma is most often found in patients 30-50 years of age and is more common in women.

Symptoms

Pain in the skin, weakness, decreased urination, fatigue, muscle wasting, intolerance to cold, joint pains, shortness of breath, kidney failure, problems swallowing, abnormal heart beats, palpitations.

Tests

A history and physical exam will be done. Lab tests to measure the immune system response of the patient are usually done. Other tests that identify damage to other body parts include: EKG, blood tests for the kidneys (electrolytes, chem 12), esophagogastroduodenoscopy (EGD), lung function test (pulmonary function test), CT scan, heart ultrasound (echocardiogram), heart catheterization, and bronchoscopy.

Treatment

There is no cure for every patient with scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat. Treatment for any organ (esophagus, lungs, heart, kidneys) damage will also be done. While still experimental (given its high rate of complications), hematopoietic stem cell transplantation is being studied in patients with severe systemic sclerosis.

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