June 8, 2012
Dear Mayo Clinic:
I am a 50-year-old woman recently diagnosed with scleroderma. What causes it, and can anything stop its progression? What medications are available, and at what point should I begin taking medication?
Scleroderma is a disease that causes the body's connective tissue and skin to harden and tighten. In some cases, only the skin is involved. But the disease can also damage other organs, including the kidneys, lungs, digestive tract and heart. The cause of scleroderma is unclear. Depending on the organs involved, medication may effectively slow or stop the disease's progression.
Scleroderma results from an overproduction of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Connective tissues are the fibers that provide the framework and support for your body.
It is not known what triggers the abnormal collagen production that leads to scleroderma, although the body's immune system appears to play a role. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease.
Scleroderma can produce a variety of symptoms, depending on which organs it affects. One common symptom of scleroderma is a heightened response to cold temperatures or to emotional distress. This condition, known as Raynaud's episode, causes blood vessels in the hands and feet to constrict. That leads to transient numbness, pain or color changes in the fingers or toes. Scleroderma can also cause persistent skin changes, such as swollen fingers and hands; thickened patches of skin, particularly on the fingers; and tight skin around the hands, face or mouth.
As a progressive disease, scleroderma typically gets worse over time. Currently, no known cure will halt the overproduction of collagen.
A variety of medications are available that may be able to control the symptoms of scleroderma. For example, angiotensin-converting enzyme, or ACE, inhibitors can be used to help relax and open blood vessels, thus relieving some kidney problems associated with scleroderma. Calcium channel blockers may decrease the symptoms of Raynaud's episodes. Several other drugs are being studied to learn if they may stop the lung inflammation that can be caused by this disease.
Some people with scleroderma may develop pulmonary hypertension. This type of high blood pressure affects the arteries in the lungs and the right side of the heart. Several medications are available that can treat pulmonary hypertension. But to be most effective, treatment must begin right away, while the condition is still in its early stages.
Treating the skin symptoms of scleroderma can be a challenge, but you can take steps that may help. Protecting yourself from cold can lower your risk of Raynaud's episodes. For example, wear mittens anytime your hands are exposed to cold — even when you reach into a freezer. When you're outside in the cold, cover your face and head and wear layers of warm clothing.
Staying active can help, too. Exercise keeps your body flexible, improves circulation and relieves stiffness. Range-of-motion exercises can help keep your skin and joints flexible. Finally, don't smoke. Nicotine causes blood vessels to constrict further and that can make symptoms of scleroderma worse. Smoking can also cause permanent narrowing of your blood vessels.
A variety of other treatments — such as stem cell transplants, the medication paclitaxel and a group of drugs known as cytokine blockers — are under investigation to see if they may be effective in reducing skin symptoms of scleroderma. Talk to your doctor to find out if any of these treatments could be right for you. Also, discuss with your doctor the best timing of treatment. In many cases, the earlier treatment begins after symptoms appear, the more effective it is in managing scleroderma.
— Thomas Osborn, M.D., Rheumatology, Mayo Clinic, Rochester, Minn.