What is scleroderma?

Scleroderma is a chronic (ongoing) condition that affects the connective tissues in your body. Connective tissue is the tissue that connects and supports your joints, muscles, skin, blood vessels and organs.

People with scleroderma usually have thickening of their skin. In some people, blood vessels and other organs are affected too.

What are the different types of scleroderma?

The 2 main types of scleroderma are:

1. localised scleroderma
2. systemic sclerosis

Localised scleroderma (morphoea)

Localised scleroderma is usually limited to your skin. This type is also called morphoea.

Symptoms include:

• itchy, swollen skin
• thickening and hardening of the skin, especially on your fingers, arms and face
• thick, discoloured patches of skin on your chest, face, arms, legs or back
• a line or band of thickened skin

Systemic sclerosis (scleroderma)

Systemic sclerosis (scleroderma), can be 'limited' or 'diffuse'. This mainly refers to:

• how much of your skin is affected
• how many of your organs are affected
• how quickly it develops

In addition to your skin, you can also have problems with your:

• blood vessels
• heart
• lungs
• kidneys
• digestive system

What are the symptoms of scleroderma?

The symptoms of scleroderma depend on:

• the type of scleroderma you have
• which part of your body is affected

Some general symptoms that can affect anyone with scleroderma are:

• tiredness
• joint pain and swelling
• muscle pain
• reflux or heartburn
• skin thickening and tightness
• swelling and stiffness of your hands
• Raynaud phenomenon — which causes your fingers or toes to change colour and feel cold, numb or painful

Symptoms can range from mild to severe.

What causes scleroderma?

Scleroderma is an autoimmune disease. It's a condition where your immune system mistakenly attacks your body.

In scleroderma, your immune system attacks healthy tissues. This causes your body to make too much collagen, making your skin and connective tissues hard and tight.

It's not clear what causes the immune system to malfunction, but it may be a combination of genes and environmental factors.

Females are affected more than males, and most people are diagnosed between the ages of 25 to 55 years.

When should I see my doctor?

See your doctor if you have symptoms of scleroderma.

How is scleroderma diagnosed?

Your doctor will ask about your symptoms, take your medical history and examine you. They will ask whether you have a family history of scleroderma.

While there is no specific test for scleroderma, your doctor may recommend tests such as blood tests.

One blood test looks for specific types of antibodies, including ANA (antinuclear antibodies). This test is often positive in people with autoimmune or connective tissue disorders.

Sometimes, a skin biopsy (small skin sample) may be done.

Lung function tests and a chest x-ray may also be recommended. This is to check if your lungs are affected.

An echocardiogram may also be recommended to check your heart.

Your doctor may refer you to a:

• dermatologist — skin specialist
• rheumatologist — a doctor who is an expert in autoimmune connective tissue disorders

How is scleroderma treated?

Unfortunately, there is no cure for scleroderma. But there are treatments available.

Your treatment will depend on your symptoms and which organs are affected.

In general, treatment for scleroderma aims to:

• manage your symptoms
• minimise damage to your body
• maintain your functioning

Treatment may involve several different healthcare professionals — you may need to see a:

• rheumatologist
• dermatologist
• physiotherapist
• occupational therapist

Treatments for Raynaud phenomenon can help with your symptoms and help prevent complications. These include self-care treatments (see below) and medicines. Self-care measures and medicines can also help treat reflux.

Medicines that suppress your immune system can help some people with scleroderma. Regular testing for complications and follow-up with your doctor are also needed.

Self-care for scleroderma

Many people with scleroderma can help manage their condition with self-care and lifestyle measures.

Self-care measures may make it easier to live with scleroderma.

• Avoid getting cold and wear gloves and socks to keep your hands and feet warm, to prevent Raynaud phenomenon.
• Keep your skin moisturised and clean to prevent dryness and infection.
• Look after your hands, feet and nails.
• Do regular range of motion exercises, especially for your hands and face, to help keep your skin and joints flexible.

Lifestyle adjustments can also help.

• Quit smoking (or vaping) and avoid exposure to smoke.
• Avoid air pollution as much as possible.
• Do some aerobic exercise, which can help improve lung function.
• Rest when you need to.
• Try to avoid stress and do relaxation exercises.

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.

Can scleroderma be prevented?

It's currently not possible to prevent scleroderma.

You can help prevent complications from Raynaud phenomenon by:

• stopping smoking
• avoiding getting cold
• reducing stress

Complications of scleroderma

Unfortunately, there are many complications of systemic sclerosis. Some of these are:

• pulmonary fibrosis (a type of lung disease)
• pulmonary hypertension (high blood pressure in the blood vessels to your lungs)
• severe high blood pressure
• kidney disease
• problems with swallowing
• constipation
• ulcers on the fingers

Resources and support

Scleroderma Australia has information brochures and local support groups for people with scleroderma.

Musculoskeletal Australia also has information and support for people with scleroderma.

You can also call the healthdirect helpline on 1800 022 222 (known as NURSE-ON-CALL in Victoria). A registered nurse is available to speak with 24 hours a day, 7 days a week.