Behçet’s disease

Introduction

Behcet’s disease - also known as Behcet’s syndrome - is a rare condition that causes inflammation of the blood vessels.

The most common symptoms of Behcet’s disease are:

  • mouth ulcers, and
  • genital ulcers.


However, Behcet’s disease can also cause inflammation of the:

  • eyes,
  • joints,
  • skin,
  • nervous system,
  • veins,
  • arteries,
  • the heart, and
  • the digestive system.


The cause of Behcet’s disease is unknown, but the immune system seems to play an important part in the development of the condition.

How common is Behcet’s disease?

Behcet’s disease seems to occur mainly in eastern Asia, the Middle East, and eastern Mediterranean.

Turkey has the highest number of cases of Behcet’s disease, where it is estimated that between 110-420 people in every 100,000 will develop it. There is also a higher than average prevalence of the condition in Japan. Behcet’s disease is rare in the UK, where it affects around 1 in 100,000.  

People of Mediterranean, Middle Eastern and Asian origin are thought to be at most at risk of developing Behcet’s disease, although it can affect all ethnic groups.

Behcet's disease is more common in men than women of Mediterranean and Middle Eastern origin, but is more common in women than men of Asian origin.

The symptoms of Bechet’s disease tend to be more severe in men.

Prognosis

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

In the most serious cases of Behcet’s disease, inflammation of the eyes can lead to permanent blindness, while inflammation of the nervous system, veins and arteries, or heart can be life-threatening. A recent study looking at Turkish patients over a 20 year period, found that almost 10% died as a result of Behcet’s disease.

There is no cure for Behcet’s disease, but it is possible to control the symptoms of the condition by using medicines that suppress the immune system (immunosuppressants).

Glossary

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
Last updated: 04 October 2011

Symptoms of Behcet's disease

Someone who has Behcet’s disease will usually begin to have symptoms between 20 and 30 years of age.

Behcet’s disease can cause a wide range of symptoms, but it is uncommon for someone with the condition to experience all of the possible symptoms. These are outlined below.

Mouth ulcers

Almost all people with Behcet’s disease have repeated episides of painful mouth ulcers. 

The ulcers tend to develop on the tongue, lips, gums and the inside of the cheek. They usually heal within 10 days, although they often return.

Genital ulcers

Like mouth ulcers, genital ulcers are also a common symptom of Behcet’s disease, occurring in an estimated 72-94% of cases.

In men, the ulcers usually appear on the scrotum, while in women they appear on the cervix, vulva or vagina. However, genital ulcers can appear anywhere in the groin region, including on the penis.

The genital ulcers are usually painful and can leave scarring. Men may also experience inflammation or swelling of the testicles, while women may find that the ulcers make sex painful.

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

Eye inflammation

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

Inflammation usually affects the uveal tract. The uveal tract is a group of connected structures inside the eye, made up of the iris (the coloured part of the eye), the ciliary body (the ring of muscle behind the iris), and the choroid (the layer of tissue that supports the retina). Inflammation of the uveal is known as uveitis.

The symptoms of uveitis can cause:

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

See the ‘useful links' section for more information about uveitis.

In the most severe cases of Behcet’s disease, inflammation of the eyes can lead to a permanent loss of vision. However, this is now far less likely if you are receiving treatment with immunosuppressants.

Skin disease

Around 80% of people with Behcet’s disease will develop skin lesions, usually on their lower limbs.

Outbreaks of acne, much like teenage acne, are also common. Skin lesions and patches of acne should heal within 14 days, but they may frequently reoccur.

Inflamed joints

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

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, it would result in a large red bump that appears to be an over-sensitive reaction.

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)

Inflammation of the veins and arteries occurs in a minority of people with Behcet’s disease. It produces redness, pain and swelling in the limbs.

In the most severe cases of vascular disease, the inflammation of the veins and arteries can result in serious and possibly life-threatening complications. The complications can include:

  • deep vein thrombosis (DVT) - a blood clot in the leg,
  • aneurysms - swelling of an artery, and
  • pericarditis - swelling of the lining of the heart.


See the ‘useful links' section for more information about these three conditions.

Gastrointestinal disease (inflammation of the stomach and intestine)

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

  • vomiting,
  • loss of appetite,
  • indigestion,
  • diarrhoea, and
  • abdominal pain.


Inflammation of the nervous system

Inflammation of the central nervous system (CNS) is the most serious type of symptom associated with Behcet’s disease.

CNS inflammation occurs in an estimated 5-10% of cases of Behcet’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,
  • partial paralysis on one side of the body,
  • changes in behaviour - people become either unusually apathetic or unusually uninhibited, and
  • loss of bowel and bladder control.

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.
Last updated: 04 October 2011

Causes of Behcet's disease

The exact cause of Behcet’s disease is unknown, but there is some evidence that both genetic and environmental factors are involved.

Genetic predisposition

The general theory about the cause of Behcet’s disease is that certain people are born with genes that make them more vulnerable to an, as yet unidentified, environmental factor, such as a virus or specific type of bacteria.

If the person is exposed to the environmental factor, something that they have inherited in the genes triggers a malfunction somewhere in their body. Most experts believe that the malfunction occurs in the immune system, causing the body to attack healthy tissue.

The scientific evidence for the genetic basis for Behcet’s disease lies in the fact that a large number of people who have developed the condition also had a specific type of gene, known as HLA B51.

Unknown environmental factor

The evidence of a possible environmental factor being involved in Behcet’s disease is that ethnic groups who are known to be at risk of developing the condition can reduce the risk by leaving their native country.

For example, rates of Behcet’s disease in immigrant Turkish communities are significantly lower than among native Turks.

Some environmental factors that have been suggested as possibly being associated with Behcet’s disease include:

  • the herpes virus,
  • the hepatitis virus,
  • bacteria, and
  • pollutants, such as industrial waste or chemicals.

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.
Last updated: 04 October 2011

Diagnosing Behcet's disease

There is no specific test for Behcet’s disease. However, your GP may recommend that you have some tests to rule out other conditions, such as arthritis, that can cause similar symptoms. The tests that you may have include:

  • blood tests,
  • urine tests, and
  • X-rays.


Behcet’s disease is diagnosed by checking for its distinctive pattern of symptoms.

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

  • repeated episodes of genital ulcers,
  • inflammation of the eye,
  • skin lesions, or
  • 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.
Last updated: 04 October 2011

Treating Behcet's disease

Immunosuppressants are the most widely used treatment for Behcet’s disease. Immunosuppressants stop the immune system from working. This interrupts the inflammation process that is the root cause of most of the symptoms associated with Behcet’s disease.

Immunosuppressants are available as a cream or lotion (topical immunosuppressants), tablet form (oral immunosuppressants), or in the form of an injection (intravenous immunosuppressants).

Topical immunosuppressants

Topical immunosuppressants are generally used to treat symptoms of:

  • mouth ulcers,
  • genital ulcers, and
  • mild cases of eye inflammation.


A cream or ointment containing corticosteroids is usually prescribed in these circumstances.

Side effects are uncommon, but long-term use of a topical immunosuppressant may result in a thinning of the top layer of your skin.

Oral immunosuppressants

Cyclosporin is the most commonly used oral immunosuppressant for treating Behcet’s disease. Cyclosporin is a systematic immunosuppressant, which means that it suppresses the whole of the immune system.

While systematic immunosuppressants can be useful in treating a wide range of symptoms in mild-to-moderate Behcet’s disease, the wholesale suppression of the immune system can cause side effects, particularly if they are taken for more than several months at a time.

Side effects of cyclosporin 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, and
  • a loss of appetite.


The long-term use of cyclosporin can also cause a rise in blood pressure and adversely affect your liver and kidney function. If you need to take cyclosporin for a significant amount of time, regular tests will be required to monitor your blood pressure and liver and kidney function.

The use of cyclosporin is not recommended for those who have symptoms of central nervous system (CNS) inflammation, as it can occasionally make those symptoms worse.

Intravenous immunosuppressants

Intravenous immunosuppressants are usually used to treat particularly severe outbreaks of the symptoms of Bechet’s disease, or when CNS inflammation has taken place. They are often used in combination with oral immunosuppressants.

The two main types of intravenous immunosuppressants are:

  • ‘traditional’ immunosuppressants - which work by suppressing the production of white blood cells, which are an important infection-fighting tool of the immune system, and
  • monoclonal antibodies  - these medicines target and kill specific proteins that are known to be involved in the inflammation process.


Traditional immunosuppressants

Examples of traditional intravenous immunosuppressants include:

  • cyclophosphamide, and
  • azathioprine.

Side effects of traditional intravenous immunosuppressants include:

  • tiredness,
  • nausea,
  • vomiting,
  • hair loss, and
  • increased vulnerability to infection.


Monoclonal antibodies

Examples of monoclonal antibodies include:

  • alemtuzumab,
  • infliximab, and
  • interferon alpha.

Common side effects of monoclonal antibodies include:

  • flu-like symptoms, such as chills, a high temperature and joint pain, and
  • fatigue.


The side effects of monoclonal antibodies may be quite bad when you take your first dose, but should improve as you take more doses and your body starts to get used to the medicine.

Less common side effects of monoclonal antibodies include:

  • nausea,
  • vomiting,
  • skin irritation at the site of the injection (changing the injection site may help with this side effect),
  • dizziness,
  • temporary hair loss,
  • ‘pins and needles’  in your hands and toes,
  • mood changes, such as depression,
  • infertility (this is usually temporary but, in some cases it can be permanent),
  • increased vulnerability to infection, and
  • changes in blood pressure and heart rhythm.


If you have any of these less common side effects, you should report them to the health professional who is treating you. Additional treatment may be available to relieve them.

If you are prescribed a course of monoclonal antibodies, you will need to be carefully monitored for any possible effects. It is likely that you will be asked to undergo regular urine, blood and blood pressure tests.

Interferon-alpha and infliximab

Recent clinical trials have found that two relatively new medicines - interferon-alpha and infliximab - seem to be successful in treating the more serious symptoms of Behcet’s disease, and can help prevent blindness and life-threatening complications.

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.
Last updated: 04 October 2011