What is scleroderma?
Scleroderma is an ongoing (chronic) disease that causes abnormal growth of connective tissue. It can affect the joints, skin, and internal organs. It is degenerative and gets worse over time. The disease can be in one area of the body. This is known as localized scleroderma. Or it may affect the whole body. This is known as systemic sclerosis. Scleroderma is more common in women.
What causes scleroderma?
Scleroderma is thought to be an autoimmune disease. This means the symptoms are caused by the body attacking its own healthy tissues. Genes play a role in the disease, but it is not passed on from parents to children. Some environmental factors may also play a role.
What are the symptoms of scleroderma?
Scleroderma can lead to scarring of the skin, joints, and internal organs. Symptoms can occur a bit differently in each person, and may include:
- Thickening and swelling of the tips of the fingers
- Pale and tingly fingers that may turn numb when exposed to cold or during emotional upset (Raynaud's phenomenon)
- Joint pain
- Taut, shiny, darker skin on large areas, that may cause problems with movement
- Spider veins
- Calcium bumps on the fingers or other bony areas
- Frozen (immobile) fingers, wrists, or elbows due to scarring of the skin
- Sores on fingertips and knuckles
- Grating noise as inflamed tissues move
- Scarring of the esophagus, leading to heartburn and trouble swallowing
- Scarring of the lungs, leading to shortness of breath
- Heart failure and abnormal heart rhythms
- Kidney disease
The symptoms of scleroderma can be like other health conditions. Make sure to see your health care provider for a diagnosis.
How is scleroderma diagnosed?
The process starts with a medical history and a physical exam. Diagnosis is based on the changes in the skin and internal organs. An antibody test may help show the type of scleroderma. Tests may also be done, such as:
Electrocardiogram (EKG or ECG). This test records the electrical activity of the heart, shows abnormal rhythms, and detects heart muscle damage. An EKG may be done to find changes in the heart muscle tissue due to scleroderma.
Echocardiogram. This test uses sound waves to create a moving image of the heart and its valves. It is done to look at the structure and function of the heart.
X-ray. This test uses a small amount of radiation to create images of internal tissues, bones, and organs. X-rays may show changes in bone, soft tissues, and organs caused by scleroderma.
How is scleroderma treated?
Treatment will depend on your symptoms, your age, and your general health. Treatment may include:
- Nonsteroidal, anti-inflammatory medications or corticosteroids, to relieve pain
- Penicillamine, to slow the skin thickening process and delay damage to internal organs
- Immunosuppressive medications, such as methotrexate
- Treatment of specific symptoms, such as heartburn and Raynaud’s phenomenon
- Physical therapy and exercise, to maintain muscle strength
- Talk with your health care providers about the risks, benefits, and possible side effects of all medications.
What are the complications of scleroderma?
With localized scleroderma, some symptoms may get better over time. However, damage to skin and other organs may be permanent. With systemic sclerosis, symptoms can over time lead to damage to the skin and cause thickened, tight skin. This can change your appearance. And it can cause movement problems. It can also cause severe kidney, lung, digestive, or heart problems. In some cases, these organ problems can lead to death.
Living with scleroderma
Systemic scleroderma is a long-term condition. It is important that you learn ways to best manage your symptoms. Learn about the disease and work with health care providers who have experience with scleroderma. Because scleroderma can affect so many systems of the body, you may need to have many specialists on your health team. Physical and occupational therapists may help you with managing your activities of daily living. Make sure that you and other team members are in regular touch with each other. If you are not able to manage your care, pick a trusted person to oversee your care. Make sure to address your emotional well-being, too. Do not hesitate to ask for help when you need it.
When should I call my health care provider?
If your symptoms get worse or you have new symptoms, let your health care provider know.
Key points about scleroderma
- Scleroderma causes abnormal growth of connective tissue. It can affect the joints, skin, and internal organs.
- Scleroderma can affect one area of the body, or affect the whole body.
- There is no cure for scleroderma. Treatment is focused on relieving pain and slowing down damage to the body.
Tips to help you get the most from a visit to your health care provider:
- Before your visit, write down questions you want answered.
- Bring someone with you to help you ask questions and remember what your provider tells you.
- At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
- If you have a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your provider if you have questions.