BEHÇET’S SYNDROME SOCIETY

Behçet’s syndrome, (now known as Behçet’s disease), is a chronic condition which happens because of 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 out-breaks 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, and 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, it is not to do with ethnic origin, gender, life-style, 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 auto-immune 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 life-span and can hope to lead close-to-normal daily lives.

No-one knows for sure how many sufferers there are in the UK but it is estimated that there are about 2 in 100,000, that is, about 2000 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 it 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.
Any other causes for these symptoms have to be ruled out first.
The symptoms do not have to occur together, but can have happened at any time.

There are levels of certainty for diagnosis:
1. International Study Group diagnostic guidelines (very strict for research purposes)
2. Practical clinical diagnosis (generally agreed pattern but not so strict)
3. ‘Suspected’ or ‘Possible’ diagnosis (incomplete pattern of symptoms)

1. International Study Group strict research level guidelines for diagnosis.

Must have mouth ulcers (any shape,size or number at least 3 times in any 12 months) Along with 2 out of the next 4 ‘hallmark’ symptoms genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men) skin lesions
(papulo-pustules, folliculitis, erythema nodosum,
acne in post-adolescents not on corticosteroids)
eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous) pathergy reaction (papule >2mm dia. 24-48hrs or more after needle-prick)

2. Practical clinical guidelines for diagnosis of patients not included in research cohorts.

Must have mouth ulcers Along with 1 of the 4 ‘hallmark’ symptoms above
And with 2 of the symptoms below arthritis/arthralgia
nervous system symptoms
stomach and/or bowel inflammation
deep vein thrombosis
superficial thrombophlebitis
cardio-vascular problems of inflammatory origin
inflammatory problems in chest and lungs
problems with hearing and/or balance
extreme exhaustion
changes of personality, psychoses
any other members of the family with a diagnosis of Behçet’s disease

3. ‘Suspected’ or ‘Possible’ diagnosis.

Usually given when someone does not have mouth ulcers
or has mouth ulcers but does not have 1 of the 4 ‘hallmark’ symptoms
but has other symptoms and signs of inflammation
and other causes for these have been ruled out

Symptom proportions for people in the UK (Survey dated 1994).

Symptom	% of people with symptom
Mouth ulcers	100
Arthitis/arthralgia	93
Genital ulcers	89
Skin lesions	86
Eye inflammation	68
Tissue reactivity (pathergy)	32
Thrombophlebitis	32
Neurological problems	31
Deep vein thrombosis (DVT)	22
Inflammatory bowel disease	28

(Other symptoms were not measured)