What is Behçet’s?

Behçet’s Syndrome (also known as Behçet’s Disease or, simply, Behçet’s) is a chronic condition resulting from disturbances in the body’s immune system.

This system, which normally protects the body against infections by producing controlled inflammation, becomes over-active and results in unpredictable outbreaks of unwanted and exaggerated inflammation. This extra inflammation particularly affects blood vessels of all sizes, including arteries and veins. As a result, symptoms occur wherever there is a patch of inflammation; this can be anywhere where there is a blood supply – from the brain down to the feet.

No-one knows why the immune system starts to behave this way in Behçet’s. It is not because of any known infections, it is not necessarily hereditary, but can sometimes run in families, and it is not thought to be related to lifestyle, age, or where someone has lived or where they have been on holiday. It is not associated with cancer, but is found more frequently in people with certain tissue-type molecules and genes, also the potential link with this is not understood. It does not follow the usual pattern for autoimmune diseases and is better considered, for now, as a disease of inflamed blood vessels (vasculitis) – a vasculitic disease.

Although Behçet’s is incurable at present, incurable does not mean untreatable. There are several ways in which the immune system can be suppressed to an appropriate level to reduce the extra inflammation, and this suppresses the symptoms.

Most of the symptoms are painful but not life-threatening. However, when the disease affects major parts of the body, such as the eyes or brain, it can cause serious consequences including blindness or strokes – but this is fortunately rare. Behçet’s symptoms typically come and go in a series of attacks (flare-ups) throughout life. The disease can often, but not always, become less severe with increasing age, where flare-ups become less aggressive and happen less frequently. Most people with Behçet’s have a normal lifespan and, with appropriate medical help, can hope to lead close-to-normal daily lives.

No-one knows for sure how many people with Behçet’s there are in the UK, but it is estimated that there are about 1 in 100,000 – that is, about 1000 people affected. It much more common in the Middle East and Asia. Because it is more prevalent in the areas surrounding the old silk trading routes, it is sometimes known as the ‘Silk Route’ disease. As yet, it is not known what triggers this disease. It is very important to have good dental hygiene, as active disease can have a bad impact on this, and poor dental hygiene can in turn result in aggravation in mouth ulcers. Some people find that paying attention to their diet can reduce their symptoms, but others don’t. Whilst there is insufficient research into this, it is good to have a sensible balanced diet.

 

  • How is Behçet’s diagnosed? There is no test for Behçet’s at the moment. It is diagnosed by specific patterns of symptoms and repeated outbreaks of them.
  • Similar Disorders There are several disorders that have patterns of symptoms that overlap with those of Behçet’s. In the absence of a test to confirm Behçet’s, this can make diagnosis quite difficult.