Behcet's disease 

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 much rarer in England, with between one and five people for every 100,000 being affected. 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 England, 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.

Due to the standard of care in England, deaths from Behçet’s disease are very rare. For example, in England and Wales during 2008, there were only two deaths as a result of Behçet’s disease.

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.