Behçet’s disease: an update on pathogenesis, diagnosis and management of vascular involvement

Abstract

The objective of this review is to summarize reports of the prevalence, clinical presentation, diagnostic methodology and treatment of vasculitic manifestations of Behçet’s disease (BD). We performed a literature search on vasculitis in BD. Articles were selected which provided insight into the pathogenesis and clinical aspects of vasculitis. Vasculitis underlies many of the clinical features of BD. Small vessel vasculitis is often found in the pathology of the mucocutaneous manifestations of BD. Large vessel vasculitis has been reported in 15-40% of BD patients. Ultrasound, angiography and tomography are applied to confirm the diagnosis when venous involvement is suspected. Endothelial dysfunction plays a role in the pathogenesis of disease. Peripheral arterial involvement in BD occurs in the form of arterial occlusion or aneurysms. Pulmonary arterial involvement is often life-threatening. The cause of cardiac vascular involvement requires an aggressive diagnostic approach. Corticosteroids and immunosuppressive agents have been used successfully in the early stage of large vessel disease and should be used as an adjunct to surgery. An increasing amount of data is available regarding the role of anti-tumor necrosis factor (TNF) agents for the treatment of BD. Anticoagulant therapy may be hazardous in patients with aneurysmal dilatation of the pulmonary vascular tree and is not effective in the treatment of venous thrombosis. Inflammation of small and large vessels is very frequent in BD. Both arteries and veins may be involved. Early recognition and appropriate management of large vessel vasculitis in BD is essential to reduce associated morbidity and mortality.