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

Abstract

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.

Ruptured popliteal artery aneurysm: An initial presentation of Behçet’s disease

“…A retrospective study from China in 2019 showed that development of aneurysms happens in the early stages of treatment naïve male BD patients, and new oral/ genital lesions or pathergy reactions can be independent risk factors for aneurysm development [9]. Interestingly it has been reported that BD patients with pulmonary artery aneurysms usually present with haemoptysis, which was not observed in our patient [9,10].…”

“…Given the complexity of disease and varied presentation, patients with BD have poor prognosis if aneurysmal lesions are identified, with pulmonary aneurysm being the most common fatal complication in men [8]. A retrospective study from China in 2019 showed that development of aneurysms happens in the early stages of treatment naïve male BD patients, and new oral/ genital lesions or pathergy reactions can be independent risk factors for aneurysm development [9]. Interestingly it has been reported that BD patients with pulmonary artery aneurysms usually present with haemoptysis, which was not observed in our patient [9,10].…”

Case report and analysis: Behçet’s disease with lower extremity vein thrombosis and pseudoaneurysm

“…Combined with aneurysm rupture, especially pulmonary aneurysm rupture, significantly increased the risk of death in BD patients ( 34 ). Pathergy, arterial occlusion, arterial stenosis, and arterial thrombosis are risk factors for aneurysm formation ( 35 ). The incidence of pseudoaneurysms is higher than that of true aneurysms in BD patients, and pseudoaneurysms are more likely to rupture ( 36 ), the formation of which is related to traumatic artery operation ( 37 ).…”

Long-term outcomes of endovascular treatment for aortic pseudoaneurysm in patients with Behçet’s disease

“…Though relatively rare in the complications of BD, 21 aneurysmal lesions are associated with high mortality due to the high risk of rupture. Zhou et al 22 found the development of aneurysmal lesions tended to occur in the early phase of BD and develop at abdominal aorta. In this study, most BAPs (58.8%, 10/17) were also observed to be sited at abdominal aorta.…”