Behcet's syndrome

--- The summary ---

Behcet's syndrome is a debilitating disorder that affects the whole
body, usually occurring in young adults. Typically, there may be mouth
ulcers, genital ulcers, arthritic joint problems, vision disturbance,
and other inflammatory-type symptoms. Since this is a recurrent
problem for which we have no cure, we use steroids to control the
severity of flare-ups, while maybe chronically using a low-dose
steroid to reduce frequency. Other anti-inflammatory and
immune-altering drugs can be useful, since lesions often becomes
steroid-resistant.

Like many other auto-immune/inflammatory disorders (of which arthritis
is one of the most well-known), there may be some genetic (familial)
tendency.

--- The technical stuff ---

This is a systemic inflammatory condition, typically affecting mucous
membranes and vascular tissues, but also affecting the central nervous
system and joints. There is a proposed viral aetiology with many
immunological features, often associated with HLA-B5 (human leukocytic
antigen).

The epidemiology suggests that it occurs in young adults, more often
occurring in Japan and the Mediterranean, and twice as likely in men
as in women.

Typical findings are:
   - recurrent oral ulcerations (aphthous type)
   - genital ulcerations
   - skin lesions
	- erythema nodosum
	- subcutaneous thrombophlebitis
	- epididymitis
   - gastrointestinal lesions
   - synovitis and other arthritic symptoms
   - meningoencephalitis
   - ocular lesions
	- acute recurrent anterior and posterior uveitis and vitritis
	     - may become chronic
	- vaso-occlusive manifestations
	  (peripheral necrotising retinal vasculitis leading to
	   haemorrages and infarction)
	     - retinitis
	     - periphlebitis
	     - aneurysms
	     - late stage changes	     
		  - secondary retinal neovascularisation
		  - rubeosis iridis
		  - glaucoma
		  - cataract
		  - retinal detachment
		  - optic atrophy

Treatment is used to control the severity of the condition, although
nothing is curative at present. Steroids are a first line of attack,
both orally and topically, but lesions often become steroid resistant
after the syndrome becomes chronic. Antimetabolites like chlorambucil
and immunosuppressants like cyclosporin are also used to control the
immune reaction. Plasma exchange is also an option.

The course is often unrelenting, with poor visual prognosis,
especially with posterior segment involvement. Visual assessment
should be carried out on a 6 monthly basis.


Last edited: 24 October 1999