Abstract

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.OverallNot progressedProgressedpn22175Aneurysm (%)21 (95.5)16 (94.1)5 (100.0)>0.999Stenosis (%)5 (22.7)4 (23.5)1 (20.0)>0.999Occlusion (%)5 (22.7)5 (29.4)0 (0.0)0.440Wall thickening (%)4 (18.2)4 (23.5)0 (0.0)0.589Thrombosis (%)11 (50.0)9 (52.9)2 (40.0)>0.999Conclusion: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.

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