Clinical course of abdominal aortic aneurysms in Behçet disease: a retrospective analysis

Arterial aneurysms are rare manifestations of Behçet Disease (BD) with high morbidity and mortality. This study aimed to investigate the clinical course of BD patients with abdominal aortic aneurysms (AAA). We retrospectively searched charts of BD patients, followed up between 1988 and 2011, to identify those with AAA with at least 6-month clinical and radiological follow-up data. Chart review revealed 12 patients (11 males) with AAA amongst 1224 patients; follow-up data from 11 patients were available. The most common symptoms were lower back and abdominal pain. The only pre-treatment complication was a spontaneous rupture. All but one patient received corticosteroid and cyclophosphamide pulses for the induction, and corticosteroid and azathioprine for the maintenance treatment; one patient received only the maintenance treatment. Two patients had surgical graft interposition, without postoperative complications. Seven patients had endovascular stenting; five of them (71.4%) showed radiological regression after 32.5 (13.4-53.8) months, while four (57%) had clinical improvement after 11.8 (0.2-29.4) months. However, one non-responsive patient developed stent infection and exsanguinated during percutaneous drainage, and one patient developed femoral artery pseudo-aneurysm at the catheter insertion site. Another patient developed a new aneurysm under the maintenance treatment. Medical treatment alone yielded radiological regression in one of two patients. Current immunosuppressive, surgical or endovascular approaches can provide clinical and radiological improvements lately in BD patients with AAA. Furthermore, complication rates seem to be high with interventional approaches. These findings suggest an unmet need for safer alternative treatments.