Scleroderma

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KEY POINTS

• Scleroderma is a disease that causes thickening and hardening of the skin and other tissues in your body.
• Exercise, occupational therapy, avoiding cold air, and good skin care can help manage your symptoms.
• Scleroderma can be treated with medicine.

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What is scleroderma?

Scleroderma is a disease that causes parts of your body to get thick and hard. It may affect the skin, joints, muscles, blood vessels, and organs such as the kidneys, heart, and lungs.

Most people have a mild form of the disease called localized scleroderma. It usually affects the skin and the muscles used for swallowing.

A more severe form of the disease is called systemic sclerosis. It affects many organs in the body. Hardening of the kidneys, heart, lungs, and digestive tract can damage or destroy the organs. In severe cases some organs eventually stop working.

Scleroderma usually develops in middle age. It is a lifelong condition, although the symptoms may come and go. Symptoms often get worse with time.

What is the cause?

The exact cause of scleroderma is not known. It appears to be an autoimmune disease. This means that your body's defense against infection (your immune system) attacks your own tissue--for example, your skin, blood vessels, or joints.

What are the symptoms?

Changes in the skin are among the most common symptoms. Your fingers and other patches of skin get hard and numb. These areas of skin may look smooth and slightly swollen. Your skin may get tight, making it hard to move your joints, especially your fingers, wrists, and elbows. You may have tightness around your mouth and eyes as well.

Another very common symptom is Raynaud's disease. Raynaud's is a problem that affects your blood vessels when you get cold or feel stressed. Your blood vessels get narrower, which means that less blood flows to your skin. When this happens, your hands, feet, ears, and nose turn pale, then blue, and then red. Your skin may throb or feel numb, tingly, or painful. Each attack may last a few minutes to an hour.

Other symptoms may include:

• Trouble swallowing pills or some foods, such as meat
• Heartburn
• Weakness
• Joint and muscle pain
• Nausea, vomiting, and stomach cramps
• Shortness of breath or trouble taking deep breaths
• Eye problems such as blurred vision and dryness

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you. Tests may include:

• Skin biopsy, which is the removal of a small sample of tissue for testing
• Blood tests
• Barium swallow, which is an X-ray taken of the esophagus and stomach after barium is swallowed. Barium is a liquid that helps your intestines show up well on the X-ray. The barium may be added to different types of food to see how well you can swallow soft or hard foods.

Other tests may be done to check organs such as your liver, kidneys, heart, or lungs.

How is it treated?

Treatment depends on your symptoms and what parts of your body are affected. Treatment doesn't cure the disease but helps to ease the symptoms. Most of the time your healthcare provider will prescribe medicine to treat your symptoms. Medicines may include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen, and naproxen, can help relieve pain and swelling. NSAIDs may cause stomach bleeding and other problems. These risks increase with age. Read the label and take as directed. Unless recommended by your healthcare provider, do not take for more than 10 days.
• Steroid medicine may be prescribed to decrease pain and swelling. It can be given as a pill, cream or ointment, or shot. Using a steroid for a long time can have serious side effects. Take steroid medicine exactly as your healthcare provider prescribes. Don’t take more or less of it than prescribed by your provider and don’t take it longer than prescribed. Don’t stop taking a steroid without your provider's approval. You may have to lower your dosage slowly before stopping it.
• Immunosuppressive medicines, which suppress or "slow down" your immune system and treat the inflammation that may be causing your symptoms. Your healthcare provider will check you regularly to see how these medicines are working.
• Vasodilators, which open up narrowed blood vessels
• Antifibrotic medicines, which prevent the buildup of tissues that cause hardening of blood vessels and organs

You may be referred to an occupational therapist if you are having trouble using your hands or drinking and swallowing. The therapist can teach you techniques and suggest tools that will help.

How can I take care of myself?

Follow the full course of treatment prescribed by your healthcare provider. In addition:

• Stay as active as you can with regular exercise or physical therapy, as recommended by your healthcare provider.
• Avoid overwork and getting too tired. Get plenty of rest.
• Avoid cold temperatures, including very cold air conditioning. Be sure to dress properly for the weather and avoid letting your skin get cold. Also, do your best to avoid stressful situations. Learn ways to manage stress, such as meditation and relaxation techniques.
• Avoid being around smoke. If you smoke, try to quit. Talk to your healthcare provider about ways to quit smoking.
• Ask your provider about heat therapy for stiff joints.
• Avoid fingersticks for blood tests.
• Use lotions, ointments, and bath oils to prevent dry and cracking skin.

Ask your healthcare provider:

• How and when you will get your test results
• How long it will take to recover
• If there are activities you should avoid and when you can return to your normal activities
• How to take care of yourself at home
• What symptoms or problems you should watch for and what to do if you have them

Make sure you know when you should come back for a checkup. Keep all appointments for provider visits or tests.

You can get more information from:

• Scleroderma Foundation
  http://www.scleroderma.org