Introduction 

Behçet's disease is a rare and poorly understood condition in which blood vessels and tissues may become inflamed (swollen).

It is sometimes referred to as an autoinflammatory disease, as certain cells of the immune system may begin this inflammation when triggered.

It is also known as Behçet's syndrome.

Signs and symptoms

The inflammation associated with Behçet's disease can affect blood vessels located within tissues throughout the body. It can therefore cause a wide range of symptoms.

Common symptoms include:

In severe causes, there is also a risk of serious and potentially life-threatening problems, such as permanent vision loss and strokes.

Read more about the symptoms of Behçet's disease.

Seeking medical advice

You should see your GP if you have a collection of persistent symptoms of Behçet's disease.

Confirming a diagnosis of Behçet's disease can be difficult because the symptoms are wide ranging and are shared with a number of other conditions.

A diagnosis will usually need to be made by a specialist based on your symptoms and by ruling out other conditions.

Read more about diagnosing Behçet's disease

What causes Behçet's disease?

The cause of Behçet's disease is unknown, although most experts believe it is an autoinflammatory condition.

An autoinflammatory condition is where the immune system – the body's natural defence against infection and illness – mistakenly attacks healthy tissue.

In cases of Behçet's disease, it is thought the immune system mistakenly attacks the blood vessels.

It's not clear what triggers this problem with the immune system, but it is very likely that both genes and environmental factors play a role.

Read more about the causes of Behçet's disease.

Who is affected?

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

It is much rarer in the UK, affecting an estimated 1,000 people in total.

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.

The symptoms of Behçet's disease can begin at any age, although they usually first appear when a person is between 20 and 30 years of age, and less commonly start after the age of 50.

How Behçet's disease is treated

There is no cure for Behçet's disease, but it is often possible to control the symptoms with medicines that reduce the inflammation in the affected parts of the body.

These medications include:

  • corticosteroids – powerful anti-inflammatory medications
  • immunosuppressants – medications that reduce the activity of the immune system
  • biological therapies – medications that target the biological processes involved in the process of inflammation

Your healthcare team will create a specific treatment plan for you depending on your symptoms.

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 (flare-ups or relapses), followed by periods where the symptoms disappear (remission).

As yet, no triggers have been identified that cause a flare-up of symptoms.

Over time, some of the symptoms can settle down and become less troublesome, although they may never resolve completely.

In the most serious cases, inflammation of the eyes can lead to vision loss. However, modern treatments can help reduce this risk when started early.

Inflammation of the nervous system, veins and arteries or heart can be life threatening, but because of the standard of care in UK, deaths from Behçet's disease are rare.

Painful mouth ulcers are a symptom of Behçet's disease 

Page last reviewed: 01/09/2014

Next review due: 01/09/2016