Management for vascular features of Behçet syndrome

Hatemi, 2018Ozguler, 2018

Vascular complications of Behçet syndrome (including vasculitis) should be managed in conjunction with a specialist. Treatment may include systemic corticosteroids plus an immunomodulatory drug, such as azathioprine, ciclosporin, or cyclophosphamide (for severe disease).

Arterial vasculitis in Behçet syndrome can lead to dilatations and aneurysms of medium- and large-sized arteries. These complications may require a combination of medical and surgical treatments, or interventional-radiological procedures. Medical treatment typically involves high-dose systemic corticosteroids plus an immunomodulatory drug, such as cyclophosphamide or a TNF inhibitor.

For venous thromboembolic events, anticoagulation using standard approaches for deep vein thrombosis is recommended; however, as people with Behçet syndrome may have arterial aneurysms, caution must be taken because of the high risk of catastrophic bleeding if an aneurysm ruptures in an anticoagulated person.