Behçet's syndrome

Behçet's has a wide range of possible symptoms but most people only have a few of these:

• Mouth ulcers – Nearly everyone with Behçet's syndrome (about 98%) has recurrent mouth ulcers. They can affect the mouth, tongue and throat and may be deep and slow to heal. Sometimes there are many tiny ulcers clustered together. If you only have occasional mouth ulcers (as many people do) it's very unlikely that you have Behçet's – this is true even if you have a relative who does have the condition.
• 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. Some men and boys also have pain or swelling in the testicles. Ulcers and boils may appear around the anus and in the groin. Neither oral nor genital ulcers in Behçet's syndrome are caused by the herpes virus. They aren't sexually transmitted or contagious, so you can't catch them from someone else.
• Skin problems – This can involve acne-like spots, boils, red patches, ulcers, spots that look like insect-bites, and lumps under the skin. The skin may become inflamed or ulcerated or appear to be infected.
• Eye inflammation – Inflammation within the eye 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 that appear to float across the field of vision), haziness or loss of vision, pain and redness in the eye.

• Fatigue – Extreme tiredness is a very common symptom.
• Joint problems – You may have aches, pains and swelling in various joints. These problems may come and go or they may persist, but they don't usually damage the joints.
• Problems with the nervous system – Some people have bad headaches. These may be caused by inflammation and you may need tests, but the headaches aren't usually a sign of anything more serious. Occasionally Behçet's causes other symptoms such as double vision, difficulty hearing, dizziness, loss of balance, fainting, and weakness or numbness in the arms or legs. Some people with Behçet's experience depression.
• Bowel problems – Many people with Behçet's syndrome have bloating and excessive wind. Behçet's sometimes causes inflammation of the bowel, leading 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). 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 affected more often than the arms. Rarely, thrombosis can occur in the blood vessels of the head, lungs or other internal organs.

It’s more common in Mediterranean countries, Turkey, the Middle East, Japan and south-east Asia and has sometimes been called the Silk Route Disease, after the ancient trade routes that ran through these areas.

Behçet’s syndrome can occur in most ethnic groups, and affects men and women of all ages, though it’s most likely to develop in your 20s or 30s. It’s a long-term (chronic) condition but may become less troublesome in later life.

It's possible that a virus or bacterial infection may trigger the condition, though no specific infection has yet been identified. It's also possible that Behçet's may be an autoimmune disease where the immune system attacks the body's own tissues. Behçet's isn't directly inherited but it's possible that genetic factors may affect your risk of developing the condition.

What tests are there?

A pathergy test measures the increased sensitivity of the skin. Your doctor will give you a small pin-prick or injection into the skin – if a red spot appears on the skin around the pin-prick then the result is positive. This doesn't mean you definitely have Behçet's but your doctor will take this result into account, along with your symptoms, when making the diagnosis.

A definite diagnosis isn't always possible, but Behçet's is likely if you have 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.

Why are blood tests used?

Although there's no test to confirm the diagnosis of Behçet's, blood tests may be done:

• to rule out other diseases
• to measure the degree of inflammation – erythrocyte sedimentation rate (ESR) and C-reactive protein
• to monitor the effect of drug treatments and to check they're not causing side-effects.

What other tests may be needed?

This varies from person to person. You may have a chest x-ray if you've not had one recently as Behçet's syndrome can occasionally cause shadows in the lungs. If you have bowel problems you may need further tests, including a telescopic examination of the bowel or stomach (endoscopy). In some specialist centres, examination of the small bowel can be carried out using a small, pill-sized camera which is swallowed (wireless capsule endoscopy). Computer tomographic (CT) and magnetic resonance imaging (MRI) scans can give more detailed images than is possible with ordinary x-rays.

Because Behçet's can affect many parts of the body, you may see and be treated by several different specialists. Usually one specialist will coordinate the treatment – often a rheumatologist or immunologist.

You may see the following specialists either for treatment or for precautionary tests:

• a gynaecologist, obstetrician, or genito-urinary medicine specialist – for genital ulcers
• a dermatologist – for skin problems
• an ophthalmologist – for eye problems
• a neurologist – for problems affecting the nervous system
• an oral medicine specialist
• a gastroenterologist – for bowel problems
• a paediatrician – for children with Behçet's syndrome.

Behçet's syndrome drugs

Colchicine tablets are often prescribed for mouth or genital ulcers, and dapsone may also be effective. There are also treatments that can be applied directly to the ulcers. These include mouth washes with steroids and antibiotics, steroid paste, and steroid sprays. Most of these are only available on prescription.

The usual treatment for moderate to severe cases of Behçet's syndrome is a group of drugs that control inflammation by suppressing the body's overactive immune system. Azathioprine is the most commonly prescribed of these drugs but ciclosporin, mycophenolate and tacrolimus are also used.

Some people need additional treatment with corticosteroids (usually prednisolone), although doctors try to limit the use of these because of their side-effects, particularly the increased risk of osteoporosis.

There is evidence that anti-TNF drugs may be effective if other treatments don't help. These have been highly successful in other inflammatory illnesses (such as rheumatoid arthritis) and are becoming more widely used in Behçet's syndrome.

Thalidomide can also be useful for treating severe ulceration, although doctors are naturally cautious about giving this to women who may become pregnant. This is because of the risk of severe birth defects.

Lastly, a drug called interferon alpha is currently under evaluation.

Painkillers may be needed in addition to the drugs mentioned above, for example to ease pain in your joints. Non-prescription types may be sufficient, but if not your doctor will be able to prescribe something stronger.

Exercise

It's important to exercise your joints and to keep up your general level of fitness. Do as much as you can but make sure you rest when you feel you need to.

Diet and nutrition

A poor diet won't cause Behçet's, but we would always recommend a balanced diet including plenty of fruit and vegetables and not too many fats and sugars for your general health.

Sex and pregnancy

The genital ulcers associated with Behçet's can sometimes make sex uncomfortable or even painful. However, they aren't sexually transmitted or contagious.

Some of the drugs used to treat Behçet's syndrome can affect fertility, and thalidomide is known to be harmful to an unborn baby. So it's important to discuss your plans with your doctor if you're thinking of having a baby – this applies to men and women with Behçet's. However, there's no reason why you shouldn't have a family and the chances of your children inheriting the condition are very low.

Living with Behçet's syndrome

Any long-term condition can affect your moods, emotions and confidence, and have an impact on your work, social life and relationships. However, treatment will help to minimize the effect. Talk things over with a friend or relative, or with your doctor if you do find your illness is getting you down.