AB0368 CLINICAL CHARACTERISTICS AND RADIOGRAPHIC OUTCOME OF VASCULAR BEHCET’S DISEASE INVOLVING AORTA AND ITS MAJOR BRANCHES
Authors: S. Lee, S. Y. Kang, Y. H. Eun, H. Kim, J. Lee, E. M. Koh, H. S. Cha
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behcet’s disease (BD) is an immune-mediated systemic vasculitis affecting any size of blood vessels. Previous studies showed that BD may involve large arterial vessels [1] but the radiographic outcome has not been well known due to its low prevalence. Objectives: To evaluate the radiographic outcome of vascular BD involving aorta and its major branches and the clinical characteristics of each prognosis group. Methods: We retrospectively analyzed the medical records of patients who visited a single-center tertiary hospital in Seoul, Korea, across a 19-year period (Jan 2002 to Dec 2020). All patients fulfilled the International Criteria for Behcet’s Disease. The patients included in this study had undergone CT angiography (CTA) of the aorta and its major branches at least twice in a 2–5 year interval and showed characteristic vascular involvements (aneurysm, stenosis, occlusion, vessel wall thickening, and thrombosis). Due to lack of the validated definition of radiographic progression of large vessel vasculitis in BD, we defined 20% changes of existing lesions or occurrence of new lesions as a radiographic progression. Results: 22 patients with BD met the inclusion criteria. 5 patients (22.7%) progressed radiographically between the initial and follow-up CTA despite low acute phase reactant levels (AUC of ESR and CRP were 13.94 mm/h and 0.32 mg/dl, respectively). Aneurysm was the most frequent form of vascular involvement followed by thrombosis (21 [95.5%] and 11 [50.0%] patients, respectively, Table 1). Patients with radiographically progressive disease had longer disease duration (1.63 vs. 6.61 years, p=0.010) at initial CTA evaluation. Other clinical characteristics, including ESR, CRP, medication use, and follow up duration, were not different between two groups (Table 2). Table 1. Radiographic characteristics of the cohort. Overall Not progressed Progressed p n 22 17 5 Aneurysm (%) 21 (95.5) 16 (94.1) 5 (100.0) &gt;0.999 Stenosis (%) 5 (22.7) 4 (23.5) 1 (20.0) &gt;0.999 Occlusion (%) 5 (22.7) 5 (29.4) 0 (0.0) 0.440 Wall thickening (%) 4 (18.2) 4 (23.5) 0 (0.0) 0.589 Thrombosis (%) 11 (50.0) 9 (52.9) 2 (40.0) &gt;0.999 Conclusion: A considerable number of vascular BD patients involving aorta and its major branches progressed radiographically despite low disease activity. Radiographically progressed patients had longer disease duration, but other clinical characteristics were not significantly different whether progressed or not. References: [1]Kural-Seyahi, Emire MD et al. The Long-Term Mortality and Morbidity of Behçet Syndrome. Medicine. 2003 Jan;82(1):60-76. Table 2. Clinical characteristics of the cohort. Overall Not progressed Progressed p n 22 17 5 Sex (Male) (%) 22 (100.0) 17 (100.0) 5 (100.0) NA Age (yr) 46.43 (7.71) 45.06 (8.06) 51.10 (4.03) 0.126 Disease duration (yr) 2.77 (3.99) 1.63 (2.66) 6.61 (5.61) 0.01 HTN (%) 5 (22.7) 4 (23.5) 1 (20.0) &gt;0.999 DM (%) 1 (4.5) 1 (5.9) 0 (0.0) &gt;0.999 Dyslipidemia (%) 6 (27.3) 5 (29.4) 1 (20.0) &gt;0.999 CKD (%) 7 (31.8) 4 (23.5) 3 (60.0) 0.321 BMI (kg/m 2 ) 42.91 (91.28) 48.23 (103.94) 24.82 (1.69) 0.626 AUC of CRP (mg/dl) 0.32 (0.37) 0.31 (0.41) 0.37 (0.12) 0.758 AUC of ESR (mm/h) 13.94 (11.40) 13.30 (12.77) 16.12 (4.66) 0.638 Immunosuppressive agent use (%) 18 (81.8) 14 (82.4) 4 (80.0) &gt;0.999 Steroid use (%) 22 (100.0) 17 (100.0) 5 (100.0) NA f/u duration (yr) 3.54 (0.80) 3.56 (0.79) 3.50 (0.92) 0.885 Data are presented as mean (SD) unless otherwise specified Disclosure of Interests: None declared