Behçet's syndrome
- Medical Eye Unit, St Thomas‘s Hospital, Lambeth Palace Road, London SE1 7EH, UK; miles.stanford@kcl.ac.uk
New treatments for an old disease
Behçet‘s syndrome, a major cause of posterior uveitis and visual loss in the Third World, has probably existed for more than 4000 years. It is a multisystem, inflammatory disorder whose principal manifestations are oral and genital aphthosis as well as inflammation of the eye, skin, and joints. The principal causes of death are from vascular and neurological involvement. No aetiological agent has yet been identified for the disease; current evidence suggests that the normal flora of mucosal tracts induce immunological hyper-reactivity in genetically predisposed individuals.
The visual prognosis in patients with Behçet‘s disease is poor, the principal cause of visual loss being consecutive inflammatory ischaemic retinal vein occlusions and macular oedema. Treatment is directed at suppressing the inflammatory response using corticosteroids and a variety of second line immunosuppressive agents. Despite different treatment regimens used in different countries the visual prognosis is much the same, with a hard core of 15–20% of patients seemingly resistant to therapy and progressing relentlessly to blindness. …
