Behçet’s disease - Treatment 

Treating Behçet’s disease  

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Increased vulnerability to infection

A drawback of taking any type of immunosuppressant is that suppressing your immune system will make you more vulnerable to infection.

You should report any symptoms of a possible infection to your GP as soon as possible because a relatively minor infection, such as a sore throat, could quickly lead to a more serious secondary infection, such as pneumonia (infection of the lungs).

Symptoms of infection include:

  • a high temperature (fever) of 38°C (100.4°F) or above
  • headache
  • aching muscles
  • diarrhoea (loose, watery stools)
  • fatigue (tiredness)

Depending on the type of immunosuppressant you are taking, you may be given antibiotics as a precaution against infection. You should also ensure that all of your vaccinations are up to date. Your GP, or care team, will be able to advise you about this.

However, you may not be able to have any vaccines that contain activated particles of viruses or bacteria. This includes:

You should avoid contact with anyone who is known to have an infection, even if it is a type of infection that you were previously immune to, such as measles or chickenpox. This is because your previous immunity to these conditions will probably be lowered (suppressed).

Once a diagnosis of Behçet’s disease has been confirmed, it is likely that you will be referred to several different specialists who have experience of treating Behçet’s disease. They will draw up a specific treatment plan for you.

As Behçet’s disease can affect many different parts of the body, the specialists 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

Immunosuppressants are the main type of treatment for Behçet’s disease. They are a type of medication that stops the immune system from working. This interrupts the inflammation process that causes most of the symptoms of Behçet’s disease.

Immunosuppressants are available as:

  • topical immunosuppressants – cream, lotion, gel or ointment
  • oral immunosuppressants – a tablet or capsule
  • intravenous immunosuppressants –an injection

Immunosuppressants are usually effective, although some types can cause side effects.

To begin with you will be prescribed an immunosuppressant that causes few side effects. If this proves to be ineffective, an alternative 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’. 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 treatment given for mouth and genital ulcers. A gel, cream, lotion or spray containing topical corticosteroids is usually recommended.

Many people find that using a steroid inhaler is effective. Steroid inhalers are commonly used to treat asthma and 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 associated with topical immunosuppressants are uncommon, but long-term use may result in a thinning of the top layer of your skin.

Read more about topical corticosteroids.

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 you take too much. It is therefore very important that you follow the recommended dose.

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 was first introduced during the 1950s to treat morning sickness, but it was withdrawn when it was discovered to cause serious birth defects.

Thalidomide is given in tablet form, and is usually taken during the evening with food.

Due to the risk of birth defects and because it can affect the quality of a man’s sperm, it is very important that you use at least one, or preferably two, reliable methods of contraception, such as a condom and the contraceptive pill, while taking thalidomide.

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)
  • 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. Different treatments are introduced one by one, starting with 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 causes 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:

  • indigestion – pain or discomfort in the upper abdomen (tummy)
  • stomach ulcer  – an open sore in the lining of the stomach

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 ciclosporine (see below)
  • intravenous immunosuppressives – such as ciclophosphamide (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, they can cause side effects, particularly if they 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 ciclosporin is not recommended for people who have symptoms of central nervous system (CNS) inflammation because it can occasionally make these symptoms worse.

Cyclophosphamide

Cyclophosphamide is an intravenous immunosuppressant that can be used to treat 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 prevents the symptoms of a headache occurring
  • symptomatic medication – which helps relieve the symptoms of a headache

Beta-blockers are a widely used type of preventative medication. Symptomatic medication includes NSAIDs and a type of medication called triptans.

Read more about treating migraine and preventing migraine.

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 back passage where it dissolves.

Common side effects of mesalazine include:

  • diarrhoea (loose, watery stools)
  • abdominal (tummy) pain
  • nausea and 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:

  • heparin
  • warfarin

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.

Read more about treating thrombosis.

Aneurysm

The recommended treatment for aneurysm will depend on how likely it is 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.

Read more about treating aneurysm.

Biological therapies

Biological therapies are a new type of medication that target the biological processes involved in the process of inflammation.

For example, there is a group of medications, which are known as tumour necrosis factor alpha inhibitors (TNFa-inhibitors), which work by targeting the antibodies known to cause much of the inflammation associated with Behçet’s disease.

Types of biological therapies that are used to treat Behçet’s disease include:

While they are usually effective, biological therapies are very expensive. For example, a one-year course of infliximab can cost between £20,000 and £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 or you are at risk of developing serious complications, such as blindness

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 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 effect pregnancy will have on the symptoms of Behçet’s disease. For example, one study found that, in around one-third of women the symptoms improved, one-third experienced a worsening of their symptoms and in the remainder of women the symptoms stayed the same.

Pregnant women 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, such as in vitro fertilisation (IVF), to conceive successfully. 

Read more about treating infertility.

Neonatal Behçet’s disease

There is also a possibility that a baby can be born with a type of Behçet’s disease that can cause ulcerations on the baby’s genitals and mouth. This type of Behçet’s disease, known as neonatal Behçet’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 Behçet’s disease and the condition usually resolves within six to eight weeks after the birth.




Page last reviewed: 22/08/2012

Next review due: 22/08/2014

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