What is scleroderma?

Scleroderma is a long-term condition affecting the connective tissue of the body. Connective tissue is the tissue that connects and supports your joints, muscles, skin and organs. People with scleroderma produce too much collagen, which makes their connective tissue, including skin, hard and tight.

Scleroderma can either be ‘localised’, which only affects the skin, or ‘diffuse’, which affects internal organs or blood vessels as well as skin. This is sometimes known as systemic sclerosis.

What are the symptoms of scleroderma?

The symptoms of scleroderma depend on the type of scleroderma you have and which part of your body is affected.

Localised scleroderma is usually limited to the skin and the tissues underneath. Symptoms include thickening and hardening of the skin, especially on the fingers, arms and face. There may be thick discoloured patches of skin on the chest, face, arms, legs or back, or a line or band of thickened tissue.

Systemic scleroderma (diffuse scleroderma) may also affect the skin, but can cause symptoms in the blood vessels, heart, lungs and kidneys, as well as the digestive system.

Small, white chalky deposits of calcium may form under the skin on the fingers, knees or elbows.

Another common symptom is Raynaud’s phenomenon, which is a blood circulation problem that causes your fingers or toes to change colour and feel numb or painful in the cold. Other symptoms of systemic scleroderma include joint pain and stiffness, pain and stiffness in the muscles, shortness of breath, fatigue, indigestion or heartburn.

What causes scleroderma?

Scleroderma is an autoimmune disease, which is a condition where the immune system mistakenly attacks the body. In scleroderma, the immune system attacks healthy tissues. This causes the body to produce excess collagen. It’s not clear what causes the immune system to malfunction, but it may be a combination of genes and environmental factors.

How is scleroderma diagnosed?

There is no specific test for scleroderma. Your doctor will ask about your symptoms, take your medical history and examine you. They may also take a blood test and a small skin sample (biopsy).

The blood test may look for specific types of antibody, including ANA (antinuclear antibodies), which are often positive in people with autoimmune or connective tissue disorders.

Lung function tests and an x-ray of the lungs may be recommended if the doctor thinks your lungs are affected.

Your doctor may refer you to a rheumatologist — a specialist doctor who is an expert in diseases that affect joints, muscles and bones, as well as autoimmune connective tissue disorders.

Scleroderma symptoms may overlap with those of other disorders, so it may take several visits to a doctor or rheumatologist before scleroderma can be diagnosed.

How is scleroderma treated?

There is no cure for scleroderma, but many people will have minimal symptoms and will be able manage their condition with simple lifestyle measures and treatments.

Treatment is aimed at managing symptoms, minimising damage to the body and maintaining mobility in your joints.

There are also medications that can suppress the immune system, which may slow down progression of the disease.

Treatment of scleroderma may involve several different healthcare professionals — as well as your GP, you may also see a rheumatologist, dermatologist (skin specialist), physiotherapist or occupational therapist.

If your organs are affected, you may be referred to a specialist, such as a kidney specialist if your kidneys are affected.

Are there any self-care measures for scleroderma?

Lifestyle changes may make it easier to live with scleroderma. These include:

• wearing gloves and socks to keep your hands and feet warm, to prevent Raynaud’s phenomenon
• avoiding cigarette smoke, as this affects blood circulation
• regular range of motion exercises, especially for the hands and face, to help keep skin and joints flexible
• aerobic exercise can help improve lung function if you have systemic scleroderma
• keeping skin moisturised and clean to prevent dryness and infection
• looking after your hands, feet and nails
• joining a support group such as those organised by Scleroderma Australia

You can also talk to your doctor or an occupational therapist about aids and equipment that can make it easier to manage day to day.