Background/Purpose: The international Chapel Hill Consensus Conference (CHCC) categorized Behçet’s disease (BD) as variable vessel vasculitis, which was defined as vasculitis that can affect vessels of any size and type. Vascular manifestations have been reported in up to 50% of patients with BD, most commonly as venous thrombosis. The aim of this study is to investigate characteristics and treatment outcomes of patients with vascular BD, particularly with large vessel involvement.

Methods: A retrospective review was performed on 2,496 patients with ICD-10 code of Behçet’s disease and/or aortic disease who visited Samsung Medical Center between 2004 and 2014. Patients who were suspected to have BD and vascular involvement were enrolled.

Results: Eighty-three patients with clinical features of BD and vascular involvement were identified. Although less than half satisfied the classic International Study Group (ISG) criteria, greater proportion fulfilled the International Criteria for BD (ICBD), and all of the patients satisfied at least “suspected BD” according to the Japanese criteria. Fifty patients had arterial lesions including 34 with aorta involvement. Another 33 patients had venous thrombosis without arterial lesions. Patients showed a male predominance (87%) and a predilection of young age with the median of 42 years old. The most prominent type of arterial lesions was aneurysm (80%) with a high frequency of pseudoaneurysms. Among the aorta, the thoracic aorta was most commonly involved and 18 patients had aortic valve regurgitation. Seventy-five (90%) patients received glucocorticoids with a median initial dose of prednisolone of 30 mg per day and 68 (82%) received immunosuppressive treatment. Half of the patients had more than one relapse after stabilization of the first vascular event. During the course, 44 patients underwent surgery and/or endovascular treatment and 60% of these patients had repeated treatment for the relapse. These included 31 aortic valve replacement on 13 patients.

Conclusion: Patients with vascular BD showed a predilection of young male and 60% of patients had arterial involvement. The aorta was affected in more than half of the patients who had arterial involvement. Aneurysmal change was frequent finding and relapse rate was high during disease course. Further studies into a practical and specialized diagnostic tool for vascular BD and optimal treatment strategies are required.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-and-treatment-outcomes-of-patients-with-behcets-disease-and-vascular-involvement/