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Behçet’s disease

Page last reviewed: 13/07/2011

Behçet's disease is a rare and poorly understood condition that causes inflammation (swelling) of the blood vessels. The inflammation often occurs is in the mouth and genitals which leads to the two most common symptoms of Behçet's disease:

  • mouth ulcers - painful, clearly defined round or oval sores that form in the mouth
  • genital ulcers

However, the inflammation can also affect blood vessels that are located throughout the body and can cause a wide variety of symptoms that can be relatively mild, such as headaches and acne, or more serious, such as vision loss or, in some cases, life-threatening, such as stroke.

How common is Behçet's disease?

Behçet's disease is sometimes referred to as 'Silk Road disease' because the condition tends to be more common in countries through which the Silk Road passes.

The Silk Road is an ancient trade route that runs from East Asia through central Asia and the Middle East, ending in the countries of the eastern Mediterranean, such as Turkey, Iran and Israel.

Out of these countries, Turkey has the highest number of cases of Behçet's disease. In some parts of the country around 420 people out of every 100,000 are affected by the condition.

Behçet's disease is rare in Ireland. People of Mediterranean, Middle Eastern and Asian origin are thought to be most at risk of developing Behçet's disease, although the condition can affect all ethnic groups, including those of northern European and Afro-Caribbean descent.

In some countries, men are much more likely to develop Behçet's disease than woman. For example, in Iran, men are 20 times more likely to develop Behçet's disease than women. However, this is not the case in Ireland, or other western European countries, where the condition affects men and women equally.

The symptoms of Behçet's disease can begin at any age, although they usually first appear when a person is between 30 and 40 years of age. The symptoms tend to be worse in men.

The cause of Behçet's disease is unknown, although most experts believe that it is an autoimmune condition. An autoimmune condition is where the immune system, which is the body's natural defence against infection and illness, mistakenly attacks healthy tissue. Other, more common autoimmune conditions include rheumatoid arthritis and lupus.

Outlook

Behçet's disease is a highly unpredictable health condition. Most people with the condition will experience episodes where their symptoms are severe followed by periods of remission where the symptoms disappear. There also seems to be no identifiable triggers that cause a flare-up of symptoms.

In the most serious cases of Behçet's disease, inflammation of the eyes can lead to vision loss. It is estimated that 1 in 4 people with Behçet's disease will experience some degree of vision loss. However, in the future it is hoped that this number will decrease following the introduction of a number of new types of medication.

Inflammation of the nervous system, veins and arteries, or heart, can often be life-threatening. A recent study that looked at Turkish patients over a 20 year period found that almost 10% died as a result of Behçet's disease. However, the pattern of Behçet's disease and its severity differ between the UK and Turkey.

There is no cure for Behçet's disease, but it is possible to control the condition's symptoms by using medicines that suppress the immune system, known as immunosuppressants.

Chronic


Chronic usually means a condition that continues for a long time or keeps coming back.

Immune system


The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.

Inflammation


Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Blood


Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.

Blood vessels

Blood vessels are the tubes in which blood travels to and from parts of the body. The three main types of blood vessels are veins, arteries and capillaries.

A common symptom of Behçet’s disease is painful mouth ulcers  

Hulusi Behçet

Behçet’s disease is named after a Turkish dermatologist (skin specialist) called Hulusi Behçet. Behçet was the first person to realise that what was previously thought to be a number of unrelated symptoms were in fact the result of a single condition.

Dr Behçet published his initial findings in 1936 and the condition has been known as Behçet’s disease ever since.

Page last reviewed: 13/07/2011

Behçet's disease can cause a wide range of symptoms, but it is rare for someone with the condition to experience all of the possible symptoms.

The symptoms of Behçet's disease are outlined below.

Mouth ulcers

Almost all cases of Behçet's disease begin with the symptoms of mouth ulcers. The ulcers have the same appearance as normal mouth ulcers but they tend to be more numerous and painful. The ulcers often develop on the:

  • tongue
  • lips
  • gums
  • inside of the cheek

The ulcers usually heal within 10 days, although they often return.

See the Health A-Z topic about Mouth ulcers for more information.

Genital ulcers

Like mouth ulcers, genital ulcers are also a common symptom of Behçet's disease, occurring in an estimated 70-95% of cases.

In men, the ulcers usually appear on the scrotum and in women they usually appear on the cervix (neck of the womb), vulva or vagina. However, genital ulcers can appear anywhere in the groin area, including on the penis.

The genital ulcers are usually painful and leave scarring in around half of all cases. Men may also experience inflammation (swelling) of the testicles and women may find that the ulcers make having sex painful.

Genital ulcers that are caused by Behçet's disease are not contagious and cannot be spread through sexual intercourse.

Skin lesions

Around 80% of people with Behçet's disease will develop skin lesions, usually on their lower limbs. A lesion is any type of unusual growth or abnormality that develops on your skin, such as a bump or a discoloured area of skin.

Outbreaks of acne, that are similar to teenage acne, are also common. Skin lesions and patches of acne should heal within 14 days, although they may frequently reoccur.

See the Health A-Z topic about Acne for further information.

Inflamed joints

Around two thirds of people with Behçet's disease will experience inflammation and swelling in their joints, with the knees, ankles and wrists often being affected. The inflammation and swelling can produce arthritis-like symptoms, such as stiffness and pain, in the affected joints.

See the Health A-Z topic about Arthritis for further information.

Eye inflammation

Inflammation of the eyes is another common symptom of Behçet's disease, occurring in an estimated 30% of cases.

Inflammation usually affects the uveal tract, which is a group of connected structures inside the eye. The uveal tract is made up of the:

  • iris - the coloured part of the eye
  • ciliary body - the ring of muscle behind the iris
  • choroid - the layer of tissue that supports the retina

Inflammation of the uveal tract is known as uveitis and the symptoms can cause:

  • painful, red eyes
  • sensitivity to light (photophobia)
  • floaters (dots that move across the field of vision)
  • blurred vision

In the most severe cases of Behçet's disease, inflammation of the eyes can lead to permanent visual impairment. However, visual impairment is now far less likely to occur if you are receiving treatment with immunosuppressants. See Behçet's disease - treatment for more information.

Pathergy (over sensitive skin)

Pathergy is a term that is used to describe skin that is particularly sensitive to injury or irritation. For example, if a needle is used to prick the skin of someone who has pathergy, a large red bump would develop that appears out of proportion to the original needle prick.

Pathergy is more common among people of Middle Eastern origin and less common in Asian and white people.

Vascular disease

Inflammation of the veins and arteries occurs in an estimated 1 in 20 people with Behçet's disease. It produces redness, pain and swelling in the limbs.

Gastrointestinal disease

Inflammation of the stomach and intestine occurs most frequently in people of Japanese origin. The inflammation can cause symptoms such as:

  • vomiting (being sick)
  • loss of appetite
  • indigestion
  • diarrhoea (loose, watery stools)
  • abdominal (tummy) pain

Inflammation of the nervous system

Inflammation of the central nervous system (CNS) is the most serious set of symptom associated with Behçet's disease. CNS inflammation occurs in an estimated 5-10% of cases of Behçet's disease, usually within five years of the initial onset of symptoms.

The symptoms of CNS are most common in men. The inflammation usually develops quickly, over the space of a few days. Symptoms can include:

  • headache
  • uncontrollable twitching or shaking
  • double vision
  • drowsiness
  • loss of balance
  • partial paralysis on one side of the body
  • changes in behaviour - people usually either become unusually withdrawn or unusually reckless
  • bladder incontinence or bowel incontinence (loss of bladder or bowel control)

Blood clots

The inflammation of the blood vessels that is associated with Behçet's disease can sometimes cause the formation of blood clots inside the blood vessels. The medical term for blood clots is thrombosis.

The most common type of blood clot to affect people with Behçet's disease is deep vein thrombosis (DVT), which affects around 1 in 20 people with the condition. DVT is where a blood clot develops in one of the deep veins of the body, usually in the legs.

Symptoms of DVT include:

  • pain, swelling and tenderness in one of your legs (usually your calf)
  • a heavy ache in the affected area
  • warm skin in the area of the clot
  • redness of your skin, particularly at the back of your leg, below the knee

While the blood clot is not immediately life-threatening itself, there is a risk that it will travel out of your leg and block the supply of blood to your lungs. This is known as a pulmonary embolism and is potentially life-threatening.

Symptoms of a pulmonary embolism include:

  • breathlessness, which may come on suddenly or gradually
  • chest pain, which may be worse when you breathe in
  • coughing up blood

Both DVT and pulmonary embolism require immediate medical treatment. If you suspect that you or someone in you care is experiencing either condition you (or they) should go to the nearest accident and emergency (A&E) department immediately.

A less common type of blood clot that is associated with Behçet's disease is cerebral venous thrombosis (CVT). CVT affects a small number (0.5-1.5%) of people with Behçet's disease. It occurs when a blood clot develops inside blood vessels that run through channels known as the dural venous sinuses, which are located between the outer and inner layer of your brain.

The blood clot can increase the pressure inside your brain and also lead to an interruption of the blood supply to the brain.

Symptoms of a CVT include:

  • severe headache - this has been described as throbbing, piercing, a band of pain or as a 'thunderclap headache', which is a very severe pain that suddenly appears out of nowhere
  • slurred speech
  • fits (seizures)
  • nausea (feeling sick)
  • vomiting (being sick)
  • deafness in one ear
  • double vision
  • stroke-like symptoms, such as muscle weakness or paralysis - unlike most strokes, both sides of the body can be affected

CVT is a type of stroke because it leads to a reduction in the blood supply to the brain. Like all cases of stroke, CVT should be regarded as a medical emergency.

Therefore, if you suspect that you or someone you know is experiencing CVT you should dial  999 immediately to request an ambulance.

Aneurysm

Inflammation of the blood vessels can cause part of the blood vessel's walls to weaken. This can result in the wall bulging outwards as a result of the pressure of the blood as it passes through the vessel.
The bulge is known as an aneurysm.

Aneurysms do not usually cause any noticeable symptoms unless the wall of the blood vessel becomes so weak that the aneurysm ruptures (splits) leading to internal bleeding and possible organ failure.

The symptoms of a ruptured aneurysm can vary depending on where in the body the aneurysm developed. Possible symptoms include:

  • dizziness
  • shortness of breath
  • mental confusion
  • loss of consciousness

Glossary

Joints
Joints are the connection point between two bones that allow movement.
Anus
The anus is the opening at the end of the digestive system where solid waste leaves the body.
Retina
The retina is the nerve tissue lining the back of the eye, which senses light and colour and sends it to the brain as electrical impulses.
Ulcers
An ulcer is a sore break in the skin, or on the inside lining of the body.
Pain
Pain is an unpleasant physical or emotional feeling that your body produces as a warning sign that it has been damaged.
Blood vessels
Blood vessels are the tubes in which blood travels to and from parts of the body. The three main types of blood vessels are veins, arteries and capillaries.
Veins
Veins are blood vessels that carry blood from the rest of the body back to the heart.
Blood
Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.
Vomiting
Vomiting is when you bring up the contents of your stomach through your mouth.
Stomach
The sac-like organ of the digestive system. It helps digest food by churning it and mixing it with acids to break it down into smaller pieces.
Swelling
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Testicles
Testicles are the two oval-shaped reproductive organs that make up part of the male genitals. They produce sperm and sex hormones.
Arteries
Arteries are blood vessels that carry blood from the heart to the rest of the body.
Constipation
Constipation is when you pass stools less often than usual, or when you are having difficulty going to the toilet because your stools are hard and small.
Diarrhoea
Diarrhoea is the passing of frequent watery stools when you go to the toilet.

Page last reviewed: 13/07/2011

The exact cause of Behçet's disease is unknown, although there is some evidence to suggest that both genetic and environmental factors are involved.

Genetic predisposition

The genetic theory about the cause of Behçet's disease is that certain people are born with genes that make them more vulnerable to an environmental factor that has yet to be identified, such as a virus or a specific type of bacteria.

It is thought that if the person is exposed to the environmental factor something that they have inherited in their genes may trigger a malfunction somewhere in their body. Most experts believe that the malfunction occurs in the immune system and causes the body to attack healthy tissue.

The scientific evidence for the genetic basis for Behçet's disease lies in the fact that a large number of people who have developed the condition have a specific type of gene called HLA B51.

Recent research carried out in 2010 has also identified two other genes (IL10 and IL23R-IL12RB2) that are relatively common in people with Behçet's disease.

Unknown environmental factor

The evidence that a possible environmental factor is involved in Behçet's disease is that ethnic groups, who are known to be at risk of developing the condition, can reduce their risk by leaving their native country. For example, rates of Behçet's disease among immigrant Turkish communities are significantly lower than among native Turks.

Similarly, the condition is virtually unheard of among Japanese immigrants to the United States of America despite the condition being quite widespread in Japan compared to most other countries.

Furthermore, there are a large number of cases of Behçet's disease that develop in people who do not have any of the genes that are associated with an increased risk of developing the condition.

Possible environmental factors include:

  • viral infections - such as the herpes simplex virus (HPV) -  the virus that causes cold sores, the hepatitis family of viruses or parvovirus B19, which causes the childhood skin condition known as slapped cheek syndrome
  • bacterial infections - such as mycobacteria which causes the lung infection tuberculosis, H. pylori which can cause stomach ulcers and streptococcal bacteria, which is a common cause of throat infections
  • exposure to chemicals that are used in agricultural, such as fertilisers and pesticides

Glossary

Tissues
Body tissue is made up of groups of cells that perform a specific job, such as protecting the body against infection, producing movement or storing fat.  
Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Bacteria
Bacteria are tiny, single-celled organisms that live in the body. Some can cause illness and disease and some others are good for you.
Genetic
Genetic is a term that refers to genes- the characteristics inherited from a family member.

Page last reviewed: 13/07/2011

Ruling out other conditions

There is no specific test for Behçet's disease. However, your GP may recommend that you have some tests to rule out other conditions, such as Crohn's disease, which is a digestive condition that can cause similar symptoms. The tests that you may have include:

Behçet's disease is diagnosed by checking for its distinctive pattern of symptoms.

A diagnosis of Behçet's disease can usually be confidently made if you have experienced at least three episodes of mouth ulcers over the pass 12 months and you have at least two of the following symptoms:

  • repeated episodes of genital ulcers
  • eye inflammation
  • skin lesions
  • pathergy (over-sensitive skin)

Glossary

Ulcers
An ulcer is a sore break in the skin, or on the inside lining of the body.
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Behçet’s Syndrome Society

A natural response to receiving a diagnosis of a complex condition such as Behçet’s disease is to find out as much as possible about the condition.

However, in Ireland this may be difficult because Behçet’s disease is so rare that even most healthcare professionals know little or nothing about it.

A good place to start to learn more about Behçet’s disease is the Behçet’s Syndrome Society (BSS) website. The BSS is the UK’s official patient support group for people with Behçet’s disease.

The BSS website has a range of information about different aspects of Behçet’s disease, a member’s forum, blogs, plus links to other useful resources.

Page last reviewed: 13/07/2011

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:

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 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.

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:

  • 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.

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.

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.

Glossary

Joint
Joints are the connection point between two bones that allow movement.
Antibiotics
Antibiotics are medicines that can be used to treat infections caused by micro-organisms, usually bacteria or fungi. For example amoxicillin, streptomycin and erythromycin.
Ulcer
An ulcer is a sore break in the skin, or on the inside lining of the body.
Pain
Pain is an unpleasant physical or emotional feeling that your body produces as a warning sign that it has been damaged.
Corticosteroids
Corticosteroid is a naturally occurring hormone produced by the adrenal gland, or a synthetic hormone having similar properties. It is used to reduce inflammation, so reducing swelling and pain.
Remission
Remission is when the symptoms of a condition are reduced (partial remission) or go away completely (complete remission).
Anaesthetic
Anaesthetic is a drug used to either numb a part of the body (local), or to put a patient to sleep (general) during surgery.
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Chronic
Chronic usually means a condition that continues for a long time or keeps coming back.

Increased vulnerability to infection

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

You should report any symptom 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 38C (100.4F) 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 contains activated particles of viruses or bacteria such as:

  • the mumps, measles and rubella (MMR) vaccine
  • the polio vaccine
  • the oral typhoid vaccine
  • the BCG vaccine (used to vaccinate against tuberculosis)
  • the yellow fever vaccine

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 suppressed (lowered).

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.

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