Behçet's disease. An insight from a vascular surgeon's point of view.

Behçet's syndrome is a multisystem inflammatory disease with unknown aetiology, vasculitis being its major pathological feature. It runs an undulating course of exacerbations and remissions with a frequency that usually abates with the passage of time. Following its first description in the medical literature in 1937, many clinical manifestations, including recurrent ulcerations, eye and urogenital lesions, pulmonary and vascular involvement with thrombus and aneurysm formation, arthritis and neurological features were reported. Various studies undertaken in two Medical Faculties of Istanbul University showed that more than 5000 patients have fulfilled three or more International Behçet's Disease Study Group Criteria and thus can be diagnosed as Behçet's Disease. A total of 142 cases with pulmonary, 30 cases with arterial (non-pulmonary) and 10 cases with cardiac involvement were demonstrated since 1978. A group of 174 neuro-Behçet's Disease patients (cerebral venous and vena caval thrombosis as the cause of intracranial hypertension, extracranial vertebral artery dissection) was evaluated. Our collected data showed that surgery with special techniques and in highly selected cases may be useful if used with adjuvant medical therapy, but might be fatal or unsuccessful in the majority. No means of aggressive surgical or interventional therapy has a role in altering the course of the pathology itself when used alone and thus medical treatment is crucial to suppress the exacerbations.