Endovascular Repair and Adjunctive Immunosuppressive Therapy of Aortic Involvement in Behçet's Disease

Aortic aneurysm is a serious problem in Behçet's disease, but open surgical therapy carries the risk of recurrent pseudoaneurysm. Here the outcomes of endovascular repair and adjunctive immunosuppressive therapy for aortic disease in Behçet's disease are presented.

This was a retrospective study. Between 2002 and 2012, nine patients with Behçet's disease (8 male, median age 41 years, range 33-60 years) were treated by endovascular stent grafting for abdominal or thoraco-abdominal aortic pseudoaneurysm.

Computed tomography angiography revealed infrarenal pseudoaneurysm in six (66.6%) patients and suprarenal pseudoaneurysm in three (33.3%). Patients received immunosuppressive therapy with oral prednisolone (60 mg/day) and cyclophosphamide (200 mg/day) for 2 weeks or more before the procedure, and intravenous hydrocortisone (200 mg/day) combined with cyclophosphamide (200 mg/day) for 3 days after the procedure. Thereafter, oral immunosuppressive therapy was continued for 2 years.

A straight tube graft was implanted in seven patients and a bifurcated graft in two patients. Two stage procedures (debranching before endovascular therapy) were performed in three patients for thoraco-abdominal aortic pseudoaneurysms. Stent grafting was successful in all patients, without any peri-operative complications. However, two patients needed abdominal exploration later: one for seroma around the graft and the other for a fistula between the duodenum and the graft. No recurrence of aneurysm was observed during a mean follow up of 40 ± 16 months. One patient died in the 15th month from a non-vascular cause.

Endovascular stent graft implantation and adjunctive immunosuppressive therapy seems to be safe and effective in the treatment of aortic involvement in Behçet's disease, but this approach needs further evaluation.