Behçet's syndrome: a multidisciplinary approach to clinical care.
Behçet's syndrome is a multisystem disorder characterized by recurrent orogenital ulceration and an occlusive vasculitis. Histologically, there is a combination of a perivascular lymphocytic infiltration with endothelial cell damage coupled with a pro-thrombotic tendency. We present a multidisciplinary approach to the management of Behçet's syndrome, and compare our findings with other published studies. Over a nine-year period, 50 patients with Behçet's syndrome were followed in a multidisciplinary combined clinic. Patients were assessed by an ophthalmologist, a rheumatologist and a specialist in oral medicine. Data on disease activity and damage were collected using a standardized proforma for each specialty. Mean age of onset was 30 years; 56% were male. Recurrent oral ulceration was the commonest manifestation and the presenting feature in 76%. The commonest second systems involved were genital mucosae and eyes. We found a larger proportion of patients with ophthalmic (80%) and central nervous system (14%) manifestations compared with many other studies. There was an association between central nervous system and thrombotic events (p<0.001). Our multidisciplinary approach allowed us to keep each system involved in Behçet's syndrome under careful review. The development of recurrent sight-threatening eye disease was unpredictable and occurred despite aggressive immunosuppression.
Whallett
A J
AJ
Heartlands and Solihull NHS Trust, Birmingham and Midland Eye Centre, and Birmingham Dental Hospital, Birmingham, UK.
Thurairajan
G
G
Hamburger
J
J
Palmer
R G
RG
Murray
P I
PI
D000293:Adolescent; D000328:Adult; D000368:Aged; D001528:Behcet Syndrome / Q000150:complications / Q000628:therapy*; D018450:Disease Progression; D005128:Eye Diseases / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology / Q000628:therapy; D019226:Oral Ulcer / Q000209:etiology / Q000628:therapy; D010348:Patient Care Team; D012871:Skin Diseases / Q000209:etiology / Q000628:therapy; D014652:Vascular Diseases / Q000209:etiology / Q000628:therapy