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Background: Vascular thrombosis in Behçet syndrome (BS) is frequent (up to 40% of the cases), and associated with high morbidity and mortality rates. Objectives: Our work aims to explore the particularities of BS patients presenting with a thrombotic event (TE), and secondly to identify the factors associated with relapse. Methods: We retrospectively conducted a single-center, descriptive and analytic study, from 2000 to 2022, enrolling all BS patients. Cases of TE, including venous thrombosis (VT) and/or arterial thrombosis (AT), were included. Statistical analyses were conducted using Jamovi and R software. Results: Among 531 BS patients, TE occurred in 136 cases (24%). The cohort consisted entirely of Moroccans, mostly men, with a ratio (M/F) of 5:1. Patients were aged 29 ± 9 years (11-50) at BS onset, and 33 ± 10 years (16-57) at TE, with a median delay of 4 years 0;25. In 32% of the cases, TE was the initial presentation of BS. Cardiovascular risk factors included mainly smoking (29%). Patients presented either VT (92.5%), AT (17.6%), or both simultaneously (13%). VT mostly consisted of DVT in the lower extremities (60%), vena cava (30%), cerebral (15%), jugular (6%), and suprahepatic veins (6%). It involved one (55%), two (27%), or multiple (16%) veins. AT affected the pulmonary (42%), femoral (21%), coronary (17%), cerebral (8%) arteries, and aorta (8%). Arterial aneurysms were common (18%), mainly observed in the pulmonary arteries (11%). Intracardiac thrombosis was also visualized (10%). Noncardiovascular manifestations were mainly oral (96%) and genital (92%) ulcers, pseudofolliculitis (36%), ocular lesions (41%), and arthralgias (22%). Biological values included median CRP of 36 mg/l and normal hemostasis parameters. Patients were treated with colchicine (98%), corticoids (98%), and immunosuppressants (80%), including cyclophosphamide and azathioprine (66%), azathioprine alone (32%), TNF-a inhibitors (5%) and anti-Il-6 (1%). Curative anticoagulation was administered with LMWH and VKA (87%) and DOACs (3%). Anti-platelet aggregation was also prescribed (9%). Six patients underwent surgery with femoropopliteal bypass (n=2), thrombectomy (n=1), stent placement (n=1), and aneurysm clipping (n=1). Post-thrombotic syndrome was common (14%), characterized by chronic venous insufficiency (8%) and venous ulcers (6%). TE relapse was observed in 25% of the cases, predominantly in men (81%), of younger age (24 ± 6) at first TE. Relapses consisted of one (55%), two (32%), or multiple episodes (13%), occurring in the same vascular system (69%), a different one (12%), or both concurrently (19%). The involved territory was the same as the initial TE (46%), different (31%), or both (23%). VT relapse (12.5%) was more common after initial lower extremity (70%) and vena cava DVT (30%), while AT relapse was less reported (7.5%), primarily in the pulmonary arteries (89%). VT relapse demonstrated a strong negative correlation with AT relapse (R=-0.8; pConclusion: Our study underscores significant associations between venous and arterial thrombosis in BS, with TE at BS onset and arterial thrombosis emerging as predictive factors of relapse. These findings offer valuable insights for the development of targeted monitoring strategies in the management of vascular thrombosis in BS. REFERENCES: 1 Tazi Mezalek, Z. et al. Vascular complications of Behçet disease. Minerva Med 112, 767–778 (2021). 2 Torgutalp, M. et al. Analysis of vascular involvement in 460 patients with Behçet's syndrome: Clinical characteristics and associated factors. Joint Bone Spine 89, (2022). 3 Bettiol, A. et al. Vascular Behçet syndrome: from pathogenesis to treatment. Nature Reviews Rheumatology 19, 111–126 (2023). Acknowledgements: NIL. Disclosure of Interests: None declared.
Chadli et al. (Sat,) studied this question.