The Clinical Features, Risk Factors, and Outcome of Aneurysmal Lesions in Behcet's Disease

OBJECTIVE: To investigate the clinical features and potential risk factors of aneurysmal lesions in Behcet's disease (BD). METHODS: We retrospectively reviewed the clinical data of BD patients with aneurysmal lesions in our institute from 1997 to 2017 and compared them with 207 BD patients without aneurysmal lesions. The treatment and outcome of these patients were also analyzed. RESULTS: Sixty-nine patients were included with 117 aneurysmal lesions. The average period between BD onset and diagnosis of aneurysmal lesion was 5.4 ± 5.5 years. Thirty-three patients (47.8%) had multiple aneurysmal lesions. Ten patients developed 20 pulmonary artery aneurysms alone. For the other 97 aortic and/or peripheral artery aneurysms in 59 patients, the most commonly affected vessels were abdominal aorta (27/97, 27.8%), coronary artery (10/97, 10.3%), and superficial femoral artery (8/97, 8.2%). Multivariate analysis revealed pathergy reaction (OR = 3.78 (1.70-8.41)), arterial stenosis or occlusion (OR = 44.12 (11.56-168.35)), and arterial thrombosis (OR = 9.27 (2.33-36.93)) as independent predictors of aneurysmal lesions in BD. With a mean follow-up of 5.5 ± 4.0 years, 40 patients (58.0%) achieved clinical improvements, 15 patients (21.7%) relapsed, and 10 patients (14.5%) died. The respective estimated cumulative 1- and 5-year relapse-free rates were 91.3% and 76.3%, and the respective estimated 1- and 5-year survival rates were 95.0% and 87.2%. CONCLUSION: Aneurysmal lesions are severe complications in BD. Pathergy reaction, arterial stenosis or occlusion, and arterial thrombosis are the risk factors of aneurysmal lesions in BD. Achieving BD remission and performing surgical or interventional procedures are both important in the treatment of these patients.