Behcet syndrome

Behçet’s syndrome, (now known as Behçet’s disease), is a chronic condition that happens because of disturbances in the body’s immune system. This system, which normally protects the body against infections by producing controlled inflammation, becomes overactive 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.

History

Behçet's syndrome is defined as a multisystemic inflammatory disease, a disease of inflamed blood vessels (vasculitis) or a vasculitic disease. It is probably first described by Hippocrates in the 5th century. The syndrome carries the name of the Turkish dermatologist Hulusi Behçet, who in 1937 described a syndrome of recurrent aphthous ulcers, genital ulcerations, and uveitis leading to blindness.

Causes

No one knows why the immune system starts to behave this way in Behcet’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 autoimmune diseases.

Treatment

Behcet’s disease is incurable at present, however this 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.

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.

Frequency

No one knows for sure how many sufferers there are in the United Kingdom but it is estimated that there are about 2 in 100,000. 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 called as the Silk Route disease.

Diagnosis

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 three 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)

International Study Group diagnostic guidelines

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. 24-48 hrs or more after needle-prick).

Practical clinical diagnosis

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 Behcet’s disease.

'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.

External links

• Behçet's Syndrome Society in the UK