Tijdschrift voor Geneeskunde vol:65 issue:12 pages:575-581
Behçet's disease: more than aphtosis.
Behçet's disease is an idiopathic systemic vasculitis with a chronic course and recurrent attacks of acute inflammation. It shows a typically geographic distribution along the old silk road. The classical triad consists of oral and genital ulcers as well as uveitis. Other clinical manifestations reflect cutaneous, articular, gastrointestinal, neurological and vascular involvement.
The etiology is not well known, though multifactorial and consisting ofgenetic, infectious, immunological and environmental factors. The pathophysiology includes vasculitis, hypercoagulability, hyperfunction aof the neutrophils and autoimmune mechanisms. The diagnosis remains clinical and based on the criteria of the International Study Group for Behçet's Disease.
The morbidity and mortality derive mainly from an ophtalmological, neurological and vascular involvement. The treatment depends on the clinical presentation and may include colchicine, corticosteroids, immunosuppressive therapy and occasionnally surgery. There is a possible role for biologics, though more evidence is needed.
This brief review article focuses on the most frequent and/or threatening disease manifestations.