Background/Purpose: To analyze the clinical features of patients with Behcet’s disease(BD) complicated with aneurysmal lesions.

Methods: We retrospectively reviewed the clinical data of patients with BD complicated with aneurysmal lesions in Peking Union Medical College Hospital from 1997 to 2017, the treatment and outcome were also studied.

Results: A total of 69 patients (56 male and 13 female) were included. The average period between BD onset and diagnosis of aneurysmal lesion was 5.4±5.5 years. A total of 120 arterial aneurysmal lesions were found, including 29 aneurysms, 68 pseudoaneurysms, 2 dissecting aneurysms, and 21 with uncertain classification. Thirty-four patients (49.3%) presented with two or more aneurysmal lesions. Abdominal aorta was the most common site of involvement (29/120), followed by pulmonary artery (20/120), femoral artery (15/120), thoracic aorta (11/20), coronary artery (10/120) and iliac artery (9/120). Swelling, pain and palpable masses in the affected artery’s area are major symptoms. Patients are more intended to have other types of vascular involvement. 48 (69.6%)patients were treated with large dose of glucocorticosteroids (prednisone at 0.8-1.2mg/kg per day, or equivalent doses) and 7 (10.1%) patients received methylprednisolone pulse therapy. Cyclophosphamide was the most commonly used immunosuppressant (60/69). Biological agents were administrated in 5 refractory cases. 40 patients received surgical therapy or interventional procedures. With a mean follow up of 3.8±3.5 years, 48 patients (69.6%) achieved clinical improvements with no newly-onset arterial lesions occurred, while 3 received a second interventional therapy, 5 died (mortality rate 7.2%).

Conclusion: Aneurysmal lesions are severe and life-threatening complications in BD patients. BD patients diagnosed with aneurysmal lesions should be further evaluated to detect possible associated vascular involvements or aneurysmal lesions at other sites. Achieving BD remission and performing surgical or interventional procedures are both important in the treatment of BD patients with aneurysmal lesions.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/aneurysmal-lesions-in-behcets-disease-a-report-of-69-cases-from-a-single-center/