This booklet is for anyone who wants to find out more about Behçet’s syndrome. You may be suffering from the condition yourself, or you may be a friend or relative of someone with Behçet’s syndrome. It explains the main facts about Behcet’s syndrome, including the main symptoms and how it is treated, and answers the most common questions about the syndrome. If you want further information, there is a list of useful addresses at the end of the booklet, as well as a glossary to explain technical terms.
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, can be inflamed with this syndrome and this can occur in many different parts of the body, as explained later in the booklet.
It is possible that a specific virus or bacteria may be the cause of the syndrome, but no such 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. There is no evidence that Behçet’s syndrome can be passed on to other people. It is not associated with cancer, nor with a specific diet, nor with any particular lifestyle (past or present). Most people with the syndrome will live a normal lifespan.
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 (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.
In 1937 Professor Hulusi Behçet, a Turkish skin specialist, published a report describing patients with recurrent mouth ulcers (ulcers which keep reappearing) who also had ulcers in the genital region and inflamed eyes. This link between mouth ulcers, genital ulcers and eye inflammation is now called Behçet’s syndrome. Since this original description, it has been discovered that the syndrome may be associated with many other symptoms.
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 men and women, and can affect all age-groups, although it usually develops in the 20s and 30s. It is a long-term problem which sometimes becomes less active 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 predispose a person to 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.
What are the main symptoms of Behçet’s syndrome?
Behçet’s syndrome may have several symptoms. The main ones are:
Nearly everyone with Behçet’s syndrome (about 98%) suffers mouth ulcers which are recurrent. 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.
Women and girls may get ulcers either in the vagina or on the vulva. In men and boys ulcers affect the scrotum and/or the penis. Apart from ulcers, some men and boys experience pain in or swelling of one or both testicles. Ulcers and boils may occur in both sexes around the anus and in the groin.
A number of different skin problems may occur. These include: spots which look like teenage acne; boils; red patches; ulcers; and lumps under or in the skin. People with Behçet’s syndrome tend to have skin which easily becomes inflamed or ulcerated or appears to be infected.
Inflammation within the eyeball is one of the most serious features of Behçet’s syndrome. It must be treated as soon as possible to avoid loss of vision. Symptoms include ‘floaters’ (dots or specks which appear to float across your field of vision), haziness or loss of vision, pain and redness in the eye. Different parts of the eye can be inflamed: at the front of the eye; around the iris; at the back of the eye; and next to the retina.
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.
It is very common for people with Behçet’s syndrome to suffer from excessive tiredness, although this common symptom may have other causes.
Many people with Behçet’s syndrome feel aches and pains in various joints, often with swelling as well. These joint problems usually come and go, but may persist. This sort of joint problem is not the same as rheumatoid arthritis or osteoarthritis, and does not usually damage the joints. (See arc booklets Rheumatoid Arthritis and Osteoarthritis.)
Problems with the nervous system
Some people with the syndrome have bad headaches. These headaches are usually not 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, faints, difficulty using the arms or legs because of weakness, numbness or unusual sensations. Some people experience depression or other psychological difficulties.
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.
Inflamed blood vessels can increase the risk of thrombosis (blood clots). 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 (see ‘Problems with the nervous system’), 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, after excluding other possible causes.
There is no single blood test or other investigation that can be used to definitely diagnose 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 deliberately in order to record the result.
Doctors feel most confident in their diagnosis of Behçet’s syndrome when someone has:
- recurrent mouth ulcers (more than 3 in a 12-month period)
plus any 2 of the following:
- genital ulcers
- typical skin problems
- typical eye inflammation
- 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 ESR and CRP) measure the amount of inflammation and can help measure how active the illness is. (See arc booklet Blood Tests and X-Rays for Arthritis.)
- Several of the drugs that are used for Behçet’s syndrome need regular blood tests to make sure they are having beneficial effects, and to check that side-effects are not happening.
What other tests may be needed?
This very much varies from individual to individual. Most people will be given a chest x-ray if they have not had one recently as Behçet’s syndrome can occasionally cause shadows in the lungs. CT and MRI scans are ways of getting more detailed images than is possible with ordinary x-rays (see Glossary). 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 your general well-being. The following treatments can help:
Improving your diet
Behçet’s syndrome is not due to a faulty diet. But a well-balanced, healthy diet including plenty of fresh fruit and vegetables is an important part of treatment. It may also help to take a daily multivitamin tablet which can be bought over the counter at a pharmacy. (See arc booklet Diet and Arthritis.)
Taking the right amount of rest and exercise
People with Behçet’s syndrome should do as much as they feel able to and 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’ 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 to ulcers elsewhere.
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. Drugs currently being used for Behçet’s syndrome include colchicine, dapsone, corticosteroids (prednisolone), and other drugs which affect the immune system. In addition painkillers of varying strength may be used. (See arc leaflet Drugs and Arthritis as well as leaflets on individual drugs).
How long will the illness last?
Behçet’s syndrome is a long-term problem. It tends to go through phases in which it is better or worse. Sometimes it will improve. 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 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 be seen by 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:
- Gynaecologist/Obstetrician/Genito-Urinary Medicine specialist (or VD/STD clinic) – for genital ulcers
- Dermatologist – for skin problems
- Neurologist – for problems affecting the nervous system
- Oral Medicine specialist/Gastroenterologist – for mouth or bowel problems
- Paediatrician – for children.
A person with Behçet’s syndrome may be sent to one of these specialists as a precautionary measure, or for specialised investigations and treatment.
Is having a family affected by Behçet’s syndrome?
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 it is important for a woman or a man with Behçet’s syndrome to discuss any plans for starting a family with her or his doctor.
Autoimmune disease – a disorder of the body’s defence mechanism (immune system) in which antibodies and other parts of the immune system attack the body’s own tissue by mistake.
CRP – C-reactive protein. Concentrations of this protein in the blood 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.
ESR – erythrocyte sedimentation rate. This test 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.
Magnetic resonance imaging (MRI) – a type of scan which uses 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.
Vasculitis – inflammation of a blood vessel resulting in changes in blood flow (see arc booklet Vasculitis).