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27 November 2009
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Scleroderma

Dr Rob Hicks

Scleroderma is an autoimmune disease, where the immune system that should protect the body attacks it instead. In this case, it causes inflammation and scarring, which eventually leaves skin thickened, muscles weakened and organs damaged.


What is scleroderma?

Scleroderma (from the Greek words for 'hard' and 'skin') targets the connective tissue found in the skin, muscles and organs. It generally starts before the age of 50 and women are more commonly affected than men.

Symptoms

There are many forms of scleroderma, but the main two are:

  • Localised scleroderma (also known as morphea) , which affects just the skin
  • Systemic sclerosis, which also affects the internal organs

Initially, the skin becomes puffy, making even the simplest tasks very difficult. Later, skin thickening makes joints tight and uncomfortable. Regular moisturising and finger exercises may help keep the skin in good condition and the joints supple.

Small deposits of calcium may appear under the skin, small red spots may appear and the skin can also be itchy.

Swallowing problems are relatively common and occur because the muscles in the gullet become thickened and stiff. Acid reflux also occurs, causing the gullet to become inflamed, which makes swallowing very painful.

When other parts of the intestines are affected, diarrhoea, bloating or constipation is often the result. Leakage of bowel motions can be a problem, too. Help and treatment are available to overcome these problems.

Poor blood flow through the lungs and inflammation means they may not function to their full capacity. In addition to reporting any breathing problems, patients should have regular tests of their lung capacity and performance to determine whether treatment is necessary to help their breathing.

Similarly, the heart can be put under pressure because of poor blood flow and fibrosis. Regular heart tests are performed to determine whether the heart is under too much strain. If this is the case, treatment can be given.

Raynaud's disease can be a problem for many of those with scleroderma. This causes spasms of the small blood vessels in response to cold, a change of temperature or stress, leading to pain and loss of sensation in the fingers.

Patients with scleroderma are also at risk of developing high blood pressure, so it's important this is checked regularly. They should also be encouraged to eat a healthy diet, not to smoke and to take regular exercise within their capabilities to help keep blood pressure under control.

Causes and risk factors

Daignosing scleroderma can be difficult as the condition can develop very slowly and symptoms vary. There is no specific test to confirm the diagnosis.

No one really knows what causes it, although it's thought that certain genes may be involved in the changes that occur around the body as the disease develops.

Treatment and recovery

There's no cure for scleroderma, but there are ways of overcoming the problems it can bring.

Disease-modifying drugs such as steroids, methotrexate and newer drugs such as infliximab aim to reverse or slow the processes that cause the damage around the body. These can be used to calm down the overactive immune system and improve blood circulation, enabling the person to get on with living as normal a life as possible.

As each person with scleroderma is affected in a different way, other treatments are used to treat problems if and when they occur:

  • Moisturisers and stretching exercises for skin tightening
  • Gloves and local heat for Raynauds phenomenom
  • Acid-suppressant drugs for acid reflux and gullet inflammation
  • Anti-hypertensive drugs for high blood pressure
  • Anti-inflammatory drugs for lung inflammation

In terms of diet, it's important to have plenty of selenium and vitamins A, C, and E, which are antioxidants and help reduce inflammation. Taking regular exercise and not smoking also protect the heart.

Advice and support

Raynaud's & Scleroderma Association
Email: info@raynauds.org.uk
Website: www.raynauds.org.uk

Scleroderma Society
Tel: 0800 311 2756
Email: kim@sclerodermasociety.co.uk
Website: www.sclerodermasociety.co.uk

This article was last medically reviewed by Dr Gill Jenkins in August 2009.


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