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Behçet’s disease

Introduction

[Original article on NHS Choices website]

Behçet’s disease is a rare and poorly understood condition that causes inflammation (swelling) of the blood vessels.

It is also known as Behçet’s syndrome.  

The inflammation often occurs 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 affect blood vessels located throughout the body and can cause a wide variety of symptoms. These can be relatively mild, such as headaches and acne, or more serious, such as vision loss. In some cases symptoms can be life-threatening, such as stroke.

Read more about the symptoms of Behçet’s disease.

There is no single test that can be used to diagnose Behçet’s disease. A diagnosis is usually made based on your symptoms and by ruling out other conditions.

What causes Behçet’s disease?

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. Rheumatoid arthritis and lupus are examples of more common autoimmune conditions.

Read more about the causes of Behçet’s disease.

Who is affected?

Behçet’s disease tends to be more common in the 'silk road' countries of the Far East, Middle East and Mediterranean, such as Turkey, Iran and Israel.

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 the condition, although it can affect all ethnic groups.

In some countries, men are much more likely to develop Behçet’s disease than women. For example, in Iran, men are 20 times more likely to develop it than women. However, this is not the case in England 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.

Treating Behçet’s disease

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

Your healthcare team will create a specific treatment plan for you depending on your symptoms. There may be several types of treatment needed as Behçet’s disease can affect many different parts of the body.

Read more about treating Behçet’s disease.

Outlook

Behçet’s disease is highly unpredictable. Most people with the condition will experience episodes where their symptoms are severe, followed by periods where the symptoms disappear (known as remission). As yet, no triggers that cause a flare-up of symptoms have been identified.

In the most serious cases of Behçet’s disease, inflammation of the eyes can lead to vision loss. It is estimated that one in four 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 be life-threatening. However, due to the standard of care in England, deaths from Behçet’s disease are very rare.


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