Your treatment plan
Once a diagnosis of Behçet’s disease has been confirmed, it is likely that you will be referred to a number of different specialists who have experience of treating Behçet’s disease. This will allow a specific treatment plan to be drawn up for you.
As Behçet’s disease can affect many different parts of the body, the specialist who are involved in your care may include:
- a dermatologist - a doctor who specialises in treating skin conditions
- a rheumatologist - a doctor who specialises in treating joint conditions
- an ophthalmologist - a doctor who specialises in treating eye conditions
- a neurologist - a doctor who specialises in treating conditions that affect the nervous system and brain
Immunosuppressants are the main type of treatment for people with Behçet’s disease. Immunosuppressants are a type of medication that stop the immune system from working. This interrupts the inflammation process that is the root cause of most of the symptoms that are associated with Behçet’s disease.
Immunosuppressants are available as:
- topical immunosuppressants - cream, lotion, gel or ointment
- oral immunosuppressants - a tablet or capsule
- intravenous immunosuppressants - in the form of an injection
Immunosuppressants are usually effective although some types can cause a wide range of side effects.
To begin with you will be prescribed an immunosuppressant that is considered to be effective in treating your symptoms but will hopefully cause a limited amount of side effects. If this proves to be ineffective, an alternative immunosuppressant that could cause a wider range of side effects may be required.
Depending on the type and severity of your symptoms you may only need to take medication when you have a ‘flare-up’ of symptoms. Alternatively, you may have to take medication on a long-term basis to prevent serious complications developing, such as vision loss.
Treatments for the different symptoms that can be caused by Behçet’s disease are described in more detail below.
Mouth and genital ulcers
Topical immunosuppressants
Topical immunosuppressants are the first line treatment for mouth and genital ulcers. A gel, cream, lotion or spray containing topical corticosteroids are usually recommended.
Many people find that using a steroid inhaler is effective. Steroid inhalers are commonly used to treat asthma and essentially consist of a small tube that is used to pump a steroid spray into the lungs,. However, rather than inhaling the steroids, you use the inhaler to spray the steroids directly onto the ulcer. A steroid mouthwash and lozenges may also be useful ways of treating mouth ulcers.
Side effects that are associated with topical immunosuppressants are uncommon, but long-term use may result in a thinning of the top layer of your skin.
See the Health A-Z topic about Topical corticosteroids for more information.
Colchicine
If your symptoms are more troublesome, you may be prescribed an additional medication called colchicine. Originally designed to treat the joint condition, gout, colchicine has subsequently proved effective in treating mouth and genital ulcers.
Colchicine is taken in tablet form and works by helping to reduce levels of inflammation on the lining of the mouth and genitals.
Common side effects of colchicine include:
- nausea (feeling sick)
- vomiting (being sick)
- abdominal (tummy) pain
These side effects may improve once your body gets used to the medication.
Colchicine is not suitable during pregnancy because there is a risk that it can cause birth defects.
It can also be very poisonous if it is taken in too high a dose. It is therefore very important that you follow the recommended dosage schedule.
Thalidomide
For the most severe cases of ulcers that do not respond to treatment, you may be prescribed a powerful oral immunosuppressant called thalidomide.
Thalidomide is a medication that was first introduced during the 1950s to treat morning sickness. However, thalidomide was withdrawn after it became apparent that it could cause devastating and sometimes life-threatening birth defects.
Thalidomide is given in tablet form, which is usually taken during the evening with food.
Due to the risk of causing birth defects and affecting the quality of a man’s sperm, it is very important that you use at least one, or preferably two, reliable methods of contraception while taking thalidomide. For example, you may decide to use a condom and the contraceptive pill.
Before being prescribed thalidomide, it is likely that you will have to sign a form to confirm that you are aware of the possible risks of birth defects and of the precautions that you need to take to prevent them.
Side effects of thalidomide include:
- nausea (feeling sick)
- vomiting (being sick)
- sleepiness - do not drive or operate machinery if you experience this side effect
- constipation (difficulty passing stools)
- loss of appetite
- headaches
- skin rashes
- numbness or tingling in your hands and feet
There is also a small, but potentially very serious, risk that you will develop a blood clot when taking thalidomide. Due to this risk, you may also be given a medication called warfarin, which helps to prevent blood clots.
Joint pain
Symptoms of joint pain in cases of Behçet’s disease are treated in much the same way as more common cases of arthritis. A stepwise approach is usually recommended with the first line treatment being the painkiller, paracetamol.
If paracetamol proves to be ineffective, one of the non-steroidal anti-inflammatory drugs (NSAIDs) group of painkillers can be used, such as naproxen or diclofenac. Alternatively, you might be prescribed a type of NSAID known as a COX-2 inhibitor, which will cause less damage to the lining of the stomach than other NSAIDs.
Common side effects that are associated with the long-term use of NSAIDs include:
For particularly severe cases of joint pain, you may be given a steroid injection directly into the affected joint.
Eye inflammation
Due to the associated risk of vision loss and blindness (in the most serious cases) the symptoms of eye inflammation should be carefully monitored by an ophthalmologist. Depending on the type and severity of your symptoms, treatments to relieve inflammation may include:
- steroid eye drops
- oral immunosuppressives - such as cyclosporine (see below)
- intravenous immunosuppressives - such as cyclophosphamide (see below)
Ciclosporin
Ciclosporin is a commonly used oral immunosuppressant for treating eye inflammation as well as other symptoms of Behçet’s’ disease, such as skin lesions.
Ciclosporin is a systematic immunosuppressant, which means that it suppresses the whole of the immune system.
While systematic immunosuppressants can be useful for treating a wide range of symptoms in mild to moderate cases of Behçet’s disease, the wholesale suppression of the immune system can cause side effects, particularly if immunosuppressants are taken for more than several months at a time.
Side effects of ciclosporin include:
- increased vulnerability to infection
- tingling and numbness in your hands and feet (this should improve with time)
- trembling in your hands and feet (this should improve with time)
- swollen, or bleeding, gums
- an unpleasant metallic taste in your mouth
- nausea (feeling sick)
- loss of appetite
The long-term use of ciclosporin can also cause a rise in blood pressure and adversely affect your liver and kidney function. If you need to take ciclosporin for a significant length of time, regular tests will be required to monitor your blood pressure and your liver and kidney function.
The use of cyclosporin is not recommended for people who have symptoms of central nervous system (CNS) inflammation because it can occasionally make such symptoms worse.
Cyclophosphamide
Cyclophosphamide is an intravenous immunosuppressant that can be used to treat more severe cases of eye inflammation where loss of vision is a concern.
Side effects of cyclophosphamide include:
- fatigue (tiredness)
- nausea (feeling sick)
- vomiting (being sick)
- increased vulnerability to infection
- hair loss - this is usually temporary and your hair should start to grow back once the treatment has been completed
Cyclophosphamide is not suitable for use during pregnancy because it can cause birth defects. You should therefore use a reliable method of contraception when taking cyclophosphamide if you are a sexually active fertile woman.
Headaches
In cases of Behçet’s disease, headaches are usually treated in the same way as migraines. This means that there are two types of medication that can be used:
- preventative medication - which are taken in an effort to prevent the symptoms of a headache occurring
- symptomatic medication - which are used to help relieve the symptoms of a headache as and when they occur
Beta-blockers are a widely used preventative medication. Symptomatic medication includes NSAIDs and a type of medication called triptans.
See the Health A-Z topic about Migraine - treatment and also Migraine - prevention for more information.
Skin lesions
Mild to moderate skin lesions can be treated with topical corticosteroids or colchine. More serious cases may require a course of cyclophosphamide (see above).
Gastrointestinal disease
A medication called mesalazine can be used to reduce the levels of inflammation inside your stomach and intestines.
Mesalazine is either taken in tablet form or as a suppository. A suppository is a capsule that you insert into your rectum (back passage) where it dissolves.
Common side effects of mesalazine include:
- diarrhoea (loose, watery stools)
- abdominal (tummy) pain
- nausea (feeling sick)
- vomiting (vomiting)
- passing wind
- headache
- joint and muscular pain
- indigestion
- itching or bleeding around your rectum
More serious cases of gastrointestinal disease may require steroid tablets or cyclosporin.
Central nervous system (CNS) inflammation
Relatively minor symptoms of central nervous system (CNS) inflammation, such as drowsiness or double vision, often get better on their own without the need for treatment.
However, more serious symptoms, such as paralysis or loss of bladder or bowel control, will usually require treatment with medication. This will usually be in the form of steroid injections or intravenous immunosuppressants, such as cyclophosphamide.
Blood clots
Blood clots can be treated using anticoagulant medications, which are medications that help to dissolve the clot. Two widely used anticoagulants are:
You may also be prescribed an antiplatelet medication which will help to thin your blood as well as helping to prevent blood clots developing in the future. Low-dose aspirin is a widely used antiplatelet medication.
See the Health A-Z topic about Thrombosis - treatment for more information.
Aneurysm
The recommended treatment for aneurysm will depend on how likely it is thought that the aneurysm may rupture. If the risk is relatively low, then making lifestyle changes, such as improving your diet, should be sufficient. However, in higher risk cases, surgery may be recommended to repair the aneurysm.
See the Health A-Z topic about Aneurysm - treatment for more information.
Biological therapies
Biological therapies are a new type of medication that directly target many of the biological processes that are involved in the process of inflammation.
For example, there are a group of medications that are collectively known as tumour necrosis factor alpha inhibitors (TNFa-inhibitors) which work by targeting the antibodies that are known to cause much of the inflammation that is associated with Behçet’s disease.
Types of biological therapies that are used to treat Behçet’s disease include:
- infliximab
- interferon alpha
- adalimumab
- etanercept
- alemtuzumab
While they are usually effective, biological therapies are usually very expensive. For example, a one year course of infliximab can cost between £20,000 to £50,000 depending on the frequency and size of the dose required.
Therefore, it is likely that your local primary care trust (PCT) will only agree to fund biological therapies if:
- other medications have been used and have proven ineffective, and
- your symptoms are severe enough to have a considerable adverse impact on your quality of life and/or you are at risk of developing serious complications, such as blindness
Common side effects of biological therapies include:
- flu-like symptoms, such as chills, a high temperature and joint pain
- fatigue (tiredness)
The side effects of biological therapies may be quite severe when you take your first dose, but they should improve as you take more doses and your body starts to adjust to the medicine.
Less common side effects of biological therapies can include:
- nausea (feeling sick)
- vomiting (being sick)
- skin irritation at the site of the injection - changing the injection site may help to ease this side effect
- dizziness
- temporary hair loss
- pins and needles in your hands and toes
- mood changes, such as depression
- infertility - which is usually temporary but can be permanent in some cases
increased vulnerability to infection
changes in blood pressure and heart rhythm
If you have any of the less common side effects listed above, you should report them to the healthcare professional who is treating you. Additional treatment may be available to help relieve them.
If you are prescribed a course of biological therapies, you will need to be carefully monitored for any possible effects. It is likely that you will be asked to have regular urine, blood and blood pressure tests.
Pregnancy and fertility
Fertility is usually unaffected in women with Behçet’s disease, but it is important that any pregnancy is planned wherever possible. This is because many of the immunosuppressants that are used to treat Behçet’s disease, such as colchine and thalidomide, can cause birth defects.
Therefore, it is recommended that you use a 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 accordingly in order to make your pregnancy as safe as possible.
However, this can take some time to achieve. For example, if you were taking thalidomide, you would need to continue to use contraception for four weeks after the course has finished.
It is difficult to predict what affect pregnancy will have on the symptoms of Behçet’s’ disease. For example, one study found that the symptoms of around a third of women improved, one third experienced a worsening of their symptoms and in the remainder of women their symptoms stayed the same.
Pregnant women who are living with Behçet’s’ disease are thought to have an increased chance of needing a Caesarean section during labour because genital ulcers can sometimes make a vaginal delivery too difficult to perform safely.
The fertility of men with Behçet’s disease can be affected. This can be the result of the condition itself or of a side effect of medication such as colchine which is known to lower sperm count. Some men may require fertility treatment to successfully conceive, such as in vitro fertilisation (IVF), which is where one sperm is injected directly into one egg to fertilise it. See Infertility - treatment and IVF for more information.
Neonatal Behcet’s disease
There is also a possibility that a baby can be born with a type of Behçet’s’ disease that can cause ulcerations of the baby’s genitals and mouth. This type of Behçet’s’ disease, known as neonatal Behcet’s disease, is very rare with only one or two cases being reported every few years.
Corticosteroids can be used to help relieve symptoms of neonatal Behcet’s disease and the condition usually resolves within six to eight weeks after the birth.
