A number of different medicines can be used to treat the various symptoms of Behçet's disease, but the main types of medicines used can be broadly divided into:
- biological therapies
Corticosteroids are powerful anti-inflammatory medicines that can be useful in reducing the inflammation associated with Behçet's disease.
Depending on the specific symptoms being treated, corticosteroids are available as:
- topical corticosteroids – applied directly to the area affected as eyedrops, creams or gels
- oral corticosteroids – these reduce inflammation throughout the body and come as tablets or capsules
Occasionally, corticosteroid injections may also be used.
Side effects depend on the form of corticosteroid you are taking. Side effects associated with topical corticosteroids are uncommon, but long-term use may lead to problems such as a thinning of your skin.
The long-term use of oral corticosteroids is associated with some potentially more serious side effects, including:
- increased appetite, leading to weight gain
- mood swings
- increased pressure in the eye (glaucoma)
- high blood pressure (hypertension)
- weak and brittle bones (osteoporosis)
If you do need to take oral corticosteroids on a long-term basis you may be given additional medicines to help treat these more serious side effects.
Immunosuppressants are a type of medicine that reduces the activity of the immune system, which in turn interrupts the inflammation process that causes most of the symptoms of Behçet's disease.
Examples of immunosuppressants used to treat Behçet's disease include azathioprine, ciclosporin, methotrexate, mycophenolate mofetil and thalidomide.
These medications are usually available as tablets, capsules and injections. Colchicine, an anti-inflammatory medicine often used for gout, may also be beneficial.
While immunosuppressants can be useful in treating a wide range of Behçet's disease symptoms, they can also cause some potentially significant side effects.
For this reason, you'll be given careful advice about potential side effects and monitoring through blood tests. This is often co-ordinated by a specialist nurse.
General side effects of these medicines can include:
- effects on blood cells and liver function (regular monitoring of blood tests may be required)
- increased risk of infections – you should report any symptoms of a possible infection to your GP or healthcare team as soon as possible
- feeling and being sick
- abdominal (tummy) pain
- hair loss, which is usually temporary
- pins and needles
- muscle cramps and weakness
Some immunosuppressants can also cause birth defects and should not be taken if you're pregnant or planning a pregnancy.
If you're taking any of these medicines, you should ensure you talk to your specialist or GP about the possible effects of your medicines on a potential pregnancy.
Biological therapies are a newer type of medicine that target the biological processes involved in the process of inflammation more selectively.
For example, 1 group of medicines called tumour necrosis factor-alpha inhibitors work by targeting the antibodies thought to cause much of the inflammation associated with Behçet's disease.
Biological therapies used to treat Behçet's disease include infliximab and interferon alpha. These may either be given directly into a vein (intravenously) or by injection under the skin (subcutaneous injection) at varying intervals.
While they're often effective, biological therapies are also very expensive. Your local hospital will usually only agree to fund biological therapies on the NHS if your symptoms are severe and other medicines have not been effective.
Biological therapies can also cause a range of side effects, including:
- increased risk of infections
- feeling sick
- muscle and joint pain
- sudden, noticeable heartbeats (palpitations)
- increased heart rate (tachycardia)
As with immunosuppressant treatment, you should report any symptoms of a possible infection to your GP or healthcare team as soon as possible.
Treating specific symptoms
The specific medicines used for Behçet's disease vary depending on the symptom being treated.
Ulcers and skin lesions
Topical corticosteroids – such as creams, lozenges, mouthwashes and sprays – are usually the first treatment recommended for mouth and genital ulcers.
Some people find that using a corticosteroid inhaler is effective. These inhalers are commonly used to treat asthma and are normally used to spray the medicine into the lungs. However, rather than inhaling the steroids, you can use the inhaler to spray the steroids directly on to an ulcer.
If you have red, tender swellings on your legs (erythema nodosum), you may be prescribed colchicine tablets to reduce the inflammation of your skin.
For severe ulcers and lesions that do not respond to other treatments, other immunosuppressant tablets or biological therapies may be recommended.
Because of the potential risk of vision loss in serious cases, any eye inflammation caused by Behçet's disease should be carefully monitored by an ophthalmologist.
Treatment for eye problems often involves taking azathioprine and corticosteroid tablets, although corticosteroid eyedrops may also be useful.
In severe cases where these treatments have not helped, mycophenolate mofetil, ciclosporin or biological therapies may also be recommended.
Daily colchicine tablets can also help by reducing the inflammation in your joints.
In severe cases where these treatments have not helped, azathioprine or biological therapies may be recommended.
A number of different medicines may be used to help reduce inflammation of the stomach and bowel caused by Behçet's disease, including corticosteroids, immunosuppressants and biological therapies.
Sometimes the inflammation in your bowel causes it to become damaged and bleed. Medical treatment is usually successful, though rarely emergency surgery may be needed to stop the bleeding and remove the affected section of bowel.
In cases of Behçet's disease, headaches are usually treated in the same way as migraines. This means there are 2 types of medicines that can be used:
- medicine to prevent the symptoms of a headache occurring, such as beta blockers
- medicine to help relieve the headache, such as NSAIDs and a type of medicine called triptans
It's not entirely clear whether blood clots associated with Behçet's disease should be treated in the same way as blood clots caused by other conditions, or whether using medicine to treat the underlying inflammation is more appropriate.
Normally, blood clots are treated with a type of medicine called an anticoagulant, which helps thin the blood and reduces the risk of the blood clot blocking the flow of blood.
However, the use of anticoagulants for Behçet's disease is controversial because the blood clots are slightly different to other clots – they tend to stick to the side of the blood vessel walls, rather than break off and travel through the bloodstream.
Using anticoagulants in Behçet's disease could also potentially increase the risk of any aneurysms rupturing and causing serious internal bleeding.
Blood clots are often treated with a combination of corticosteroid or immunosuppressant tablets to reduce the inflammation in the blood vessels. Anticoagulants are only used if you've been screened to make sure there are no aneurysms.
Aneurysms caused by Behçet's disease are normally treated with a combination of corticosteroid and immunosuppressant tablets, or infusions of cyclophosphamide to reduce inflammation in the affected blood vessel and stop the aneurysm getting worse.
Surgery or procedures may also be considered. The aneurysm may be repaired or bypassed using small tubes called stents, or it can be blocked off. This is often combined with medical treatment to reduce or prevent the risk of recurrence.
Central nervous system inflammation
Relatively minor symptoms of central nervous system inflammation, such as double vision, may get better on their own without the need for treatment.
However, more serious symptoms, such as paralysis and behavioural changes, usually require treatment with medicine. This will often be in the form of corticosteroid or immunosuppressant injections, or biological therapies.
Pregnancy and fertility
Fertility is usually unaffected in women with Behçet's disease, but it's important that any pregnancy is planned where possible. This is because many of the medicines used to treat the condition, such as thalidomide, can cause birth defects.
It's recommended that you use at least 1 reliable method of contraception until you decide that you want to have a baby. You should discuss your plans to have a baby with your care team, who will be able to adjust your treatment plan to make your pregnancy as safe as possible.
There's little evidence that having Behçet's disease increases your risk of pregnancy-related complications, although there's a very small chance of your baby being born with a temporary condition called neonatal Behçet's disease.
The fertility of men with Behçet's disease may be affected. This can be the result of the condition itself or of a side effect of some of the immunosuppressant medicines. Colchicine may temporarily lower sperm count, but this often improves when treatment is stopped.
Where fertility is affected, further investigation – and, in some cases, fertility treatment such as in vitro fertilisation (IVF) – may be needed to conceive successfully.
Read more about treating infertility.
Neonatal Behçet's disease
There's a possibility that a baby can be born with a type of Behçet's disease that can cause ulcers on the baby's genitals and mouth. This type of Behçet's disease, known as neonatal Behçet's disease, is extremely rare, with only 1 or 2 cases being reported every few years.
Corticosteroids can be used to help relieve symptoms of neonatal Behçet's disease. The condition usually resolves within 6 to 8 weeks after the birth.