Behçet's syndrome: a multidisciplinary approach to clinical care

QJM. 1999 Dec;92(12):727-40. doi: 10.1093/qjmed/92.12.727.

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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Behcet Syndrome / complications
  • Behcet Syndrome / therapy*
  • Disease Progression
  • Eye Diseases / etiology
  • Eye Diseases / therapy
  • Female
  • Humans
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Nervous System Diseases / etiology
  • Nervous System Diseases / therapy
  • Oral Ulcer / etiology
  • Oral Ulcer / therapy
  • Patient Care Team
  • Skin Diseases / etiology
  • Skin Diseases / therapy
  • Vascular Diseases / etiology
  • Vascular Diseases / therapy