Patient Information - Raynaud’s, Systemic Sclerosis & Associated Disorders


Patient Information - Topics - Raynaud’s, Systemic Sclerosis & Associated Disorders

'The information contained in this patient information sheet is not a substitute for medical advice or
treatment, and the Society recommends consultation with your doctor or health care professional'

1. What is Raynaud's?

Raynaud's is a condition where the blood supply to the extremities, usually the fingers and toes, but occasionally the nose or ears, is interrupted. During an attack the affected part first becomes white and dead looking, then turns blue as the tissues use up the oxygen and finally bright red as the arteries relax and fresh blood rushes in. In some cases only one or two of these classical colour changes may occur. Raynaud's phenomenon is due to spasm of the small blood vessels of the fingers or toes and is usually induced by exposure to cold although sometimes can occur in response to changes in temperature and to emotional changes such as stress and anxiety. Other stimuli can occasionally provoke an attack, e.g. drugs, tobacco smoke, and hormones. Raynaud's phenomenon is more common in females with an overall prevalence in the population of 10%. Anyone of any age can suffer from Raynaud's, but teenage women are affected more commonly. This may decline in severity after the menopause.
Primary Raynaud's Disease is the term used for Raynaud's phenomenon when there is no clear underlying cause or associated condition. It can vary from a very mild form, being little more than a nuisance, to a severe form requiring treatment.
Secondary Raynaud's Syndrome is associated with some other disease or external influence. Conditions associated with Raynaud's phenomenon include connective tissue diseases such as systemic sclerosis, treatment with certain drugs, occupational disease such as hand arm vibration syndrome, and thoracic outlet syndrome.

 

2. What is Systemic Sclerosis?

This is a disease of the connective tissue, which as the name implies holds our bodies together. Therefore not only the skin can be affected, but also internal organs. The majority of sufferers have the mild form where there is limited skin involvement, usually of the hands and feet, becoming stiff and shiny. The gullet often becomes affected making eating and swallowing difficult. Some patients also form tiny deposits of calcium under the skin (calcinosis) which can cause ulceration. In the more severe form, called diffuse scleroderma, wide areas of skin and internal organs such as the lungs, bowel heart and kidneys are affected. Localised skin disease (scleroderma) can be divided into two types: Morphea and linear scleroderma. Morphea is the name given to localised patches of hardening of the skin. Linear scleroderma develops in childhood and may affect the growth of a limb, and is usually limited to one area. Unlike morphea, linear scleroderma tends to involve deeper layers and can affect the mobility of underlying joints. The incidence of Raynaud’s phenomenon in systemic sclerosis is 95%.

 

3. How are they diagnosed?

Most commonly Raynaud’s phenomenon is diagnosed from the history, and occasionally on examination if seen during an attack. Blood tests may help, as can examining the small blood vessels at the base of the nail (nail fold capillaroscopy).

 

4. Is it hereditary?

There is no evidence at present that either Raynaud's or systemic sclerosis are directly inherited. There is however a genetic predisposition, so that the chances of being affected are greater if a relative has the problem.

 

5. How can I help myself?

There are several things you can do which may help. The most important is to stop smoking, take regular exercise and keep warm.
Smoking. If you are a smoker you must make a sincere and determined effort to give up completely. Tobacco is harmful as it causes the blood vessels to constrict, decreasing the blood flow to the finger tips. The best way to give up is to choose a day when you are going to stop completely rather than trying to cut down gradually. If you do have trouble giving up please ask your doctor who can put you in touch with a support group.
Eating for warmth. Eating and drinking can help you keep warm. Try to eat lots of small meals to maintain your energy, high protein foods, milk, meat, fish, and fresh vegetables are best. Hot meals and plenty of hot drinks, especially before retiring are essential.
Exercise. Gentle exercise will help your circulation. Try to avoid sitting for long periods. Get up and walk around the room, moving arms and legs to maintain the circulation. Do not however let your fingers or toes get cold. In cold weather take exercise indoors.
Clothing. Tight clothing should be avoided as this may restrict blood flow. Hands and feet should always be adequately covered. A scarf should be used to keep the face warm in cold weather and a hat and several layers of clothing should be used to keep the head and trunk warm. Feet are especially prone to cooling, therefore a good thick pair of socks is essential. Wet shoes and clothes should be changed as soon as possible.

 

6. What about treatment?

Your GP or specialist may prescribe a vasodilator such as nifedipine, which is a drug that relaxes the blood vessels. Occasionally, your specialist may feel an operation, called a sympathectomy, may be of benefit. This involves either cutting or destroying the nerves that cause the arteries to constrict. This operation is more successful for Raynaud's of the feet than the hands. Your specialist will explain these procedures to you. Heated gloves and socks may benefit some patients with Raynaud’s phenomenon and can be prescribed.

 

7. Associated conditions

Vibration White Finger. Those who work with vibrating tools have a tendency to develop Raynaud's especially if the vibration is coarse and of low frequency. This can become permanent even after the work has stopped. VWF is an industrial disease which may be eligible for compensation.
Chilblains. These usually appear on the extremities-fingers, toes, and ears. The skin may first become itchy, the red, swollen and very tender to touch. Chilblains occur as a result of defective circulation on exposure to cold. Clothing that rubs should be avoided.
Rheumatoid Arthritis. Arthritis affects the lining of the joints. This lining produces a fluid that lubricates the joint and when affected by rheumatoid arthritis it becomes inflamed and swollen. More fluid is produced causing a red, painful swollen joint. About 10% of rheumatoid arthritis sufferers have Secondary Raynaud's.
Systemic Lupus Erythematosis. This is characterised by a rash sometimes seen on both cheeks and the bridge of the nose, and chronic inflammation of the blood vessels and connective tissues of the body. There is associated tiredness, joint pain, mouth ulcers, hair loss and Raynaud's.
Erythromyalgia. This is a chronic disorder characterised by persistent warmth, pain and redness, mainly affecting the feet and lower legs. The majority of sufferers also experience Raynaud's symptoms.
Chemical or drug induced. Some chemicals at work (vinyl chloride) or drugs such as Beta Blockers, migraine tablets or the oral contraceptive may aggravate Raynaud's, therefore, if you are prescribed any medicines and you experience Raynaud's type symptoms, check with your GP who may be able to alter your medication.

 

8. What is the prognosis?

People who develop Raynaud's as teenagers often have a form that is benign and will disappear with age. Unfortunately this is not true in all cases, and sometimes Raynaud's does persist. There is no cure for systemic sclerosis at present but there are many effective treatments available to alleviate specific symptoms.

More details can be obtained from: The Raynaud's Association, 112 Crewe Rd., Alsager, Cheshire ST7 2JA. Tel 01270-872776.

The Vascular Society is grateful to Mr Simon Parvin and members of the Audit and Research Committee for writing this information leaflet.

March 2006


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