Behçet's Syndrome
An Information Booklet
Introduction
This booklet is for anyone who wants to find out more about Behçet's syndrome – or Behçet's disease, as it is also known. You may be suffering from the condition yourself, or you may have a friend or relative with Behçet's syndrome. We explain the main facts about Behçet's syndrome, including the main symptoms and how it is treated. We aim to answer the most common questions which people ask about the syndrome, but further information can be obtained from the organizations listed at the back of the booklet. We have also included a glossary of medical terms (like vasculitis). We have put these in italics when they first appear in the booklet.
What is a syndrome and how was Behçet's syndrome discovered?
A syndrome is a group of symptoms (that is, what you experience) and signs (that is, what the doctor finds by examining you). Doctors tend to talk of a syndrome rather than a disease when the cause linking the different features is not known. Although many doctors now refer to 'Behçet's disease', others believe that it may not be a single disease with a single cause. For this reason we prefer to use the term 'syndrome' throughout this booklet, but you will often hear the term 'Behçet's disease' used instead.
In 1937 Professor Hulusi Behçet, a Turkish skin specialist, published a report describing patients with recurrent mouth ulcers who also had ulcers in the genital region and inflammation of the eyes. This combination of mouth ulcers, genital ulcers and eye inflammation became known as Behçet's syndrome. Since this original description it has been discovered that the syndrome may be associated with many other symptoms (see Figure 1).
What is Behçet's syndrome?
We do not know what causes Behçet's syndrome, or even if there is just one cause. Whatever the cause, the result is that affected tissues become inflamed. Small blood vessels, particularly, become inflamed (this is known as vasculitis) and this can cause symptoms in many different parts of the body, as explained later in this booklet. Although doctors distinguish this type of vasculitis from other forms (which are described in the arc booklet 'Vasculitis') some of the symptoms may be similar.
It is possible that a specific virus or bacterium may be the cause of the syndrome, but no specific infection has yet been clearly identified. It is also possible that Behçet's syndrome is an autoimmune disease in which the immune system attacks the body's own tissues, but this is not yet certain, and the disease is different from other autoimmune diseases. There is no evidence that Behçet's syndrome can be passed on to other people. It is not associated with cancer, or with a specific diet, or with any particular lifestyle (past or present). Most people with the syndrome will live a normal lifespan.
How common is Behçet's syndrome?
Behçet's syndrome is rare in the UK. We do not know exactly how many people in the UK have Behçet's syndrome, but there are probably about two thousand. The condition is more common in Mediterranean countries, Turkey, the Middle East, Japan and south-east Asia. Because Behçet's syndrome is more common in these regions, the illness has been called the Silk Route Disease, after the ancient trade route for silk which ran through these areas.
Behçet's syndrome can occur in most ethnic groups, and we still do not know to what extent ethnic background increases or reduces the chances of getting it. Behçet's syndrome occurs in males and females, and can affect all age groups, although it usually develops in the 20s and 30s. It is a long-term (chronic) problem which sometimes becomes less troublesome in later life.
Does Behçet's syndrome run in families?
It is unusual for more than one member of a family to get Behçet's syndrome. It is not hereditary but there are genetic factors that may increase the likelihood of a person developing the condition. It is possible for relatives of someone with Behçet's syndrome to have mouth ulcers, but this does not mean that they will get Behçet's syndrome. Mouth ulcers are very common in the general population, and occur in as many as 1 person in 5 (20%).
What are the main symptoms of Behçet's syndrome?
Behçet's syndrome may have several symptoms, many of which are painful. The main ones are:
Mouth ulcers
Nearly everyone with Behçet's syndrome (about 98%) suffers recurrent mouth ulcers. They may reappear occasionally or frequently. These ulcers can affect anywhere around the mouth, tongue and throat and are often painful. They are called 'minor' if they are superficial and heal rapidly, and 'major' if they are deep and slow to heal. Sometimes there are many tiny ulcers clustered together, in which case they are described as 'herpetiform' (from the Greek 'herpes', meaning 'to creep').
Genital ulcers
Women and girls may get ulcers on the vulva, in the vagina or on the cervix. In men and boys ulcers affect the scrotum and/or the penis. Apart from ulcers, some men and boys experience pain or swelling in one or both testicles. Ulcers and boils may occur in both sexes around the anus and in the groin. Neither oral nor genital ulcers in Behçet's syndrome are caused by the herpes virus.
Skin problems
A number of different skin problems may occur. These include spots which look like teenage acne, boils, red patches, ulcers, spots which look like flea- or insect-bites, and lumps under or in the skin. People with Behçet's syndrome tend to have skin which easily becomes inflamed or ulcerated or which appears to be infected.
Eye inflammation
Inflammation within the eyeball is one of the most serious features of Behçet's syndrome. The inflammation may be at the front or the back of the eye, around the iris, or next to the retina (see Figure 2). It must be treated as soon as possible to avoid loss of vision. Symptoms include 'floaters' (dots or specks which appear to float across the field of vision), haziness or loss of vision, pain and redness in the eye.
IMPORTANT NOTE: If you experience any of the eye symptoms above, you should contact your doctor promptly so that an eye clinic can see you as soon as possible.
Fatigue
It is very common for people with Behçet's syndrome to suffer from excessive tiredness.
Joint problems
Many people with Behçet's syndrome feel aches and pains in various joints, often with swelling as well (see Figure 3). These joint problems usually come and go, but they may persist. This sort of joint problem is not the same as rheumatoid arthritis or osteoarthritis, and does not usually damage the joints.
Problems with the nervous system
Some people with the syndrome have bad headaches. These headaches are not usually serious, but may be due to inflammation and may need extra tests and further treatment. Occasionally Behçet's syndrome can cause other symptoms such as double vision, difficulty hearing, giddiness, loss of balance, fainting, and difficulty using the arms or legs because of weakness, numbness or unusual sensations. Some people experience depression or other psychological difficulties.
Bowel problems
Many people with Behçet's syndrome have bloating (feeling as though the stomach is blown up) and excessive wind. Behçet's syndrome can sometimes cause inflamed bowels, which may lead to diarrhoea and abdominal pain with blood and/or mucus in the stools.
Thrombosis
Inflamed blood vessels can increase the risk of blood clots (thrombosis). This sort of thrombosis is not the same as that which causes heart attacks. When it involves veins near to the skin surface they become painful, hot and red. Thrombosis in deeper veins or arteries leads to pain and swelling of the affected limb. The legs are more commonly affected than the arms. Rarely, thrombosis can occur in the blood vessels of the head, lungs or other internal organs.
How can I be sure that Behçet's syndrome is the correct diagnosis?
The symptoms and signs of Behçet's syndrome can sometimes be confused with other illnesses. So it is important to exclude these other diseases before deciding that the problem is Behçet's syndrome. Doctors will look at the combination of the typical features and try to exclude other possible causes.
There is no single blood test or other investigation that can be used to confirm a diagnosis of Behçet's syndrome, but the presence of what is called 'pathergy' is a useful clue. Pathergy is a measure of the increased sensitivity of the skin which occurs in Behçet's syndrome. This often shows as a red spot appearing where there is a minor injury to the skin, such as a blood-test, pin-prick, insect-bite, sting or scratch. The pathergy test is when a doctor gives you a small pin-prick or injection into the skin in order to record the result (see Figure 4).
The diagnosis of each individual depends on the exact circumstances. However doctors feel most confident in their diagnosis of Behçet's syndrome when someone has:
- recurrent mouth ulcers (more than three in a 12-month period)
plus any two of the following:
- genital ulcers
- typical skin problems
- typical inflammation in the eye
- a positive pathergy test.
The presence of additional features (e.g. arthritis, thrombosis) may increase the likelihood that the diagnosis is correct.
Why are blood tests used?
Although there is no blood test that can be used to confirm the diagnosis of Behçet's syndrome, there are several reasons for having blood tests:
- Blood tests are needed to rule out other diseases that can be confused with Behçet's syndrome.
- Some blood tests, such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) test, measure the amount of inflammation and can help measure how active the illness is. In Behçet's syndrome the results of these tests act as a guide but do not always accurately reflect the underlying illness or how you feel.
- Several of the drugs that are used for Behçet's syndrome require regular blood tests to make sure they are having beneficial effects, and to check that they are not causing side-effects. These tests include the full blood count, urea and electrolytes (U&E), and liver function tests.
What other tests may be needed?
This varies a great deal from person to person. Some people may be given a chest x-ray if they have not had one recently as Behçet's syndrome can occasionally cause shadows in the lungs. Those with bowel problems may need further tests, including a telescopic examination of the bowel or stomach ('endoscopy') and/or barium x-rays. Computer tomographic (CT) and magnetic resonance imaging (MRI) scans are ways of getting more detailed images than is possible with ordinary x-rays. These scans may be needed to look further into particular symptoms.
How is Behçet's syndrome treated?
At the moment, there is no cure for Behçet's syndrome. But treatment can control the severity and frequency of the symptoms and improve general well-being. The following measures and treatments can all help:
Improving your diet
Behçet's syndrome is not caused by a faulty diet. But a well-balanced, healthy diet including plenty of fresh fruit and vegetables is an important part of treatment.
Taking the right amount of rest and exercise
People with Behçet's syndrome should do as much as they feel able to, but allow for plenty of rest when needed. It is important to exercise your joints as much as pain or discomfort will allow and to keep up your general level of fitness.
Treating the ulcers directly
There are several treatments which are designed to be applied directly to the ulcers (that is, 'local' or 'topical' treatments). These include mouth washes with steroids and antibiotics, steroid paste, and steroids that can be sprayed on developing ulcers, using dispensers designed for asthmatics. Most of these can only be obtained with a prescription from your doctor. Steroids can also be prescribed to apply to the eye and also to genital and skin ulcers.
Drug treatment
There are many drugs that can be used to control Behçet's syndrome. Doctors aim to match the strength of the drug with the seriousness of the problem, since the chances of side-effects are generally higher with the more powerful drugs. (See arc leaflet 'Drugs and Arthritis' as well as leaflets on individual drugs).
Drugs currently being used for Behçet's syndrome include colchicine, dapsone, corticosteroids (usually prednisolone), and other drugs which affect the immune system (azathioprine, mycophenolate mofetil, ciclosporin, tacrolimus). Patients with severe ulcers or skin problems may benefit from thalidomide. Thalidomide is a very useful drug for immune-related diseases but doctors are extremely cautious about giving thalidomide to women if there is any chance of pregnancy. In addition there are new forms of treatment (e.g. anti-TNF agents) which have been successful in other illnesses and which are under evaluation for Behçet's syndrome.
Painkillers of varying strength may be necessary in addition to the drugs mentioned above.
How long will the illness last?
Although Behçet's syndrome is a long-term problem, it does not usually affect how long you live. The illness tends to go through phases in which sometimes it is better than others. The right treatment may prevent new symptoms from appearing and control the existing ones. Because of the many possible problems that can occur, most people with Behçet's syndrome should have a regular review in a specialist clinic, or at least have easy access to such a clinic.
How will the illness affect my quality of life?
Although treatment will help to reduce how often symptoms occur and how severe they are, it does not usually get rid of them entirely. Thus many people find that their quality of life is affected. Recent research into the quality of life for people with Behçet's syndrome has shown that it can stop people doing as much as they want to, or as much as they used to, and can be an important factor in decisions about career choices and family planning. It can affect moods, emotions and confidence, and, like many other long-term illnesses, it can have an impact on work, social activities and relationships.
How many different specialists do I need to see?
Since it can affect many different parts of the body, someone with Behçet's syndrome may need to see several different specialists. Ideally, one specialist will coordinate the treatment, and this role is often taken on by a rheumatologist or immunologist, often in conjunction with an ophthalmologist (who deals with the eyes). Other specialists often involved include:
- a gynaecologist/obstetrician/genito-urinary medicine specialist (or STD/sexual health clinic) – for genital ulcers
- a dermatologist – for skin problems
- a neurologist – for problems affecting the nervous system
- an oral medicine specialist – for mouth problems
- a gastroenterologist – for bowel problems
- a paediatrician – for children with Behçet's syndrome.
A person with Behçet's syndrome may be sent to one of these specialists as a precautionary measure, or for specialized investigations and treatment.
Will I be able to have a family?
There is no reason why anyone with Behçet's syndrome should not aim to have children. The chances of children inheriting Behçet's syndrome are very low. However, some of the drugs used to treat the syndrome can affect sperm, eggs, fertility or even the baby. So if you have Behcet's syndrome and are thinking of starting a family it is important that you discuss your plans with your doctor. This applies to men and women.
Glossary
Anti-TNF – drugs which block the action of a
protein in the blood called tumour necrosis factor (TNF) which causes
inflammation when present in excessive
amounts. Adalimumab, etanercept and infliximab are all anti-TNF
drugs.
Autoimmune disease – a disorder of the body's defence mechanism (immune system), in which antibodies and other components of the immune system attack the body's own tissues.
Barium x-ray – an x-ray which is taken after a barium meal or enema. Barium is a white liquid which coats the inside of, for example, the bowel so that the outline shows up on the x-ray.
C-reactive protein (CRP) – a protein in the blood which can be measured to test for inflammation or disease activity.
Computer tomographic (CT) scan – a type of scan which records images of sections or 'slices' of the body using x-rays. These images are then transformed by a computer into cross-sectional pictures.
Erythrocyte sedimentation rate (ESR) – a test which shows the level of inflammation in the body. It is based on the speed at which red blood cells (erythrocytes) settle in a sample of blood.
Full blood count – a blood test used to measure haemoglobin (found in red blood cells), white blood cells and platelets. This test is frequently carried out to monitor drug treatment.
Liver function tests – blood tests used to check the healthy functioning of the liver. They are frequently performed to monitor drug treatment.
Magnetic resonance imaging (MRI) – a type of scan which uses radio waves in a strong magnetic field to build up pictures of the inside of the body. It works by detecting water molecules in the body's tissue which give out a characteristic signal in the magnetic field.
Urea and electrolytes (U&E) – a blood test that mainly monitors the kidneys. This is frequently carried out to monitor drug treatment.
Vasculitis – inflammation of a blood vessel resulting in changes in blood flow. (Note that the vasculitis in Behçet's syndrome is different from other forms of vasculitis, which are described in the arc booklet 'Vasculitis'.)
Useful addresses
The Arthritis Research Campaign (arc)
PO Box 177
Chesterfield
Derbyshire S41 7TQ
Phone: 0875 850 5000
www.arc.org.uk
As well as funding research, we produce a range of free information booklets and leaflets. Please contact the address above for a list of titles or, on this web site, contact Publications for People with Arthritis.
Arthritis Care
18 Stephenson Way
London NW1 2HD
Phone: 020 7380 6500
Helpline (freephone): 0808 800 4050
www.arthritiscare.org.uk
Offers self-help support, a helpline service, and a range of leaflets on arthritis.
Behçet's Syndrome Society
43 Parkgate Crescent
Hadley Wood, Barnet
Hertfordshire EN4 0NW
Phone: 0845 130 7328
Helpline: 0845 130 7329
www.behcets.org.uk
Provides information and advice for people with Behçet's syndrome.
Information on drugs
Separate arc leaflets are available on many of the drugs used for arthritis and related conditions. We would recommend that you read the relevant leaflets for more detailed information about your medication.
