Thrombotic manifestations in pediatric behcet syndrome: a multicenter comparative study from the eurofever registry

Seminars in Arthritis and Rheumatism(2024)

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Abstract
Introduction Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8 to 21% pediatric BS patients, even if the real prevalence is still largely unknown. Objectives To report clinical features and outcomes of pediatric BS patients with thrombosis and to compare the demographic and clinical characteristics of BS patients with and without thrombosis. Methods Retrospective data collection of BS patients with thrombosis (T+) included in the EUROFEVER registry. BS patients without thrombosis (T-), belonging to the same rheumatology units, were matched in a 2:1 ratio. Results 37 T+ were compared to 74 T- patients. At onset, ICBD criteria fulfillment was higher in the T- group (p=0.015). Caucasian patients were more often T-, Turkish patients were more frequent in T+ group (p=0.002). At onset, pustulosis was most frequently observed in the T- (p<0.001) as well as gastrointestinal symptoms (p<0.001) and ocular involvement (p=0.022). Neurological symptoms were more often described in T+ (p=0.034). As for T+, thrombosis was reported at BS presentation in 8/37 (21.6%). For the T+ e patients who developed thrombosis later, oral aphthosis (p=0.003), genital aphthosis (p=0.014) were more frequently observed at BS onset, while pustulosis (p=0.005) and fever (p=0.043) coexisted with thrombosis. Thrombosis was mainly venous (26/37,70.3%), involving the cerebral sinuses (21/37, 56.8%). After thrombosis, 35/37 (94.6%) T+ patients received an immunomodulatory treatment compared with 16/29 (55.2%) pre-thrombosis. A recurrence was reported in 6/31(19.4%). Conclusion Thrombosis was reported at BS presentation one fifth of cases. Pustulosis and fever were more frequently concomitant to thrombosis. Sinus veins were the most frequent site.
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Key words
Behçet syndrome,thrombosis,child
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