Outcomes of vascular intervention and use of perioperative medications for nonpulmonary aneurysms in Behçet disease

Background Aneurysms attributable to Behçet disease (BD) are not common but are associated with a poor prognosis because of a high risk of rupture. Special considerations are required for vascular intervention, because the intense local inflammation may increase complications. The aim of this study was to assess the outcome of operative intervention and the use of perioperative medical therapy for aneurysms in patients with BD. Materials and methods We reviewed retrospectively the medical records of patients with BD admitted to Peking Union Medical College Hospital between January 1995 and January 2015. Results Among 874 patients diagnosed with BD, 176 patients had vascular involvement, of whom 59 had arterial aneurysms. Thirty-six patients with 51 arterial aneurysms underwent operative intervention. The 51 primary operative interventions included 20 open operations and 31 endovascular interventions. Eleven (22 %) recurrent aneurysms developed in 10 patients and 5 (10%) thrombosis occurred in 5 patients. Of the 19 endovascular stents placed for aortic aneurysms, 1 type I endoleak, 1 graft occlusion, 3 recurrent aneurysms, and 1 recurrent aneurysmal rupture occurred. Among the revascularization procedures for extremity arteries, there were more complications with endovascular intervention than with open surgery. The cumulative risk of recurrent aneurysmal formation at the operative site was significantly less in patients treated with operative intervention combined with use of immunosuppressants than those treated with operative intervention alone ( P = .01). Conclusion In patients with BD, an endovascular approach is feasible and effective for aortic aneurysms, whereas bypass surgery appears to provide better outcomes for extremity arterial aneurysms than placement of endovascular stents. The administration of corticosteroids combined with cyclophosphamide perioperatively decreases the cumulative risk of recurrent aneurysm.