| Literature DB >> 10581336 |
A J Whallett1, G Thurairajan, J Hamburger, R G Palmer, P I Murray.
Abstract
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.Entities:
Mesh:
Year: 1999 PMID: 10581336 DOI: 10.1093/qjmed/92.12.727
Source DB: PubMed Journal: QJM ISSN: 1460-2393