What is Behçet’s disease?
Behçet’s syndrome (now known as Behçet’s disease) 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 produces unpredictable outbreaks of unwanted and exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result symptoms occur wherever there is a patch of inflammation; this can be anywhere where there is a blood supply.
No-one knows why the immune system starts to behave this way in Behçet’s disease. It is not because of any known infections, it is not hereditary, and it is not to do with ethnic origin, gender, lifestyle, age, where someone has lived or where they have been on holiday. It is not associated with cancer, and links with tissue-types (which are under investigation) are not certain. It does not follow the usual pattern for autoimmune diseases. At the moment it is defined as being a disease of inflamed blood vessels (vasculitis) – a ‘vasculitic’ disease.
Although Behçet’s disease 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. They come and go in a series of attacks (‘flare-ups’) throughout life. The disease does not ‘burn itself out’, but sometimes it can level off and the flare-ups become less aggressive and happen less frequently. Most people with Behçet’s disease have a normal lifespan and can hope to lead close-to-normal daily lives.
No-one knows for sure how many people with Behçet’s disease there are in the UK, but it is estimated that there are about 1 in 100,000 – that is, about 1000 people. It is much less rare in the Middle East and in Asia. Because it seems to be prevalent in the areas surrounding the old silk trading routes, it is sometimes known as the ‘Silk Route’ disease.
- How is Behçet’s disease diagnosed? There is no test for Behçet’s disease 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 disease. In the absence of a test to confirm Behçet’s, this can make diagnosis quite difficult.
- BSS factsheets All the factsheets listed are available free of charge from the Society, and many can be downloaded here.
- Headache in Behçet’s disease Headache is one of the most continual and troublesome symptoms in Behçet’s disease. Some patients are lucky and hardly ever get headaches; others have daily chronic severe throbbing headaches that are very disabling.