Thrombotic manifestations in pediatric Behçet syndrome: a multicenter comparative study from the EUROFEVER registry

Mv Mastrolia, C Matucci-Cerinic, S Ozen, O Kasapcopur, C Gaggiano, I Koné-Paut, L Cantarini, P Dusser, Ü Kaya-Akça, M Yildiz, J Brunner, G Filocamo, R Gallizzi, A Insalaco, S Pastore, Donato Rigante, J Sanchez-Manubens, E Tsitsami, N Ruperto, M GattornoG Simonini

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Introduction: Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8–21 % 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 in one fifth of cases. Pustolosis and fever were more frequently concomitant to thrombosis. Sinus veins were the most frequent site.
Lingua originaleEnglish
pagine (da-a)1-7
Numero di pagine7
RivistaSeminars in Arthritis and Rheumatism
Volume2024
DOI
Stato di pubblicazionePubblicato - 2024

Keywords

  • Behçet's disease

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