Thrombotic Manifestations in Pediatric Behçet Disease Patients: A Multicentre Comparative Study from EUROFEVER Registry

Maria Vincenza Mastrolia¹, Caterina Matucci-Cerinic², Seza Ozen³, Ozgur Kasapcopur⁴, Carla Gaggiano⁵, Isabelle Kone-Paut⁶, Luca Cantarini⁵, Perrine Dusser⁷, Ümmüşen Kaya-Akça⁸, Mehmet Yildiz⁹, Juergen Brunner¹⁰, Giovanni Filocamo¹¹, Romina Gallizzi¹², Antonella Insalaco¹³, Serena Pastore¹⁴, Donato Rigante¹⁵, Judith Sanchez-Manubens¹⁶, Elena Tsitsami¹⁷, Marco Gattorno¹⁸ and Gabriele Simonini¹⁹, ¹NEUROFARBA Department, University of Florence, Florence, Italy, ²DINOGMI, University of Genoa; IRCCS Istituto Giannina Gaslini, UOC Rheumatology and autoinflammatory diseases, Genova, Italy, ³Hacettepe University Medical Faculty, Ankara, Turkey, ⁴Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, ⁵Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy, ⁶Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAIA, Bicêtre Hospital, APHP, University of Paris Saclay, Paris, France, ⁷Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, ⁸Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁹Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Instanbul, Turkey, ¹⁰Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ¹¹Pediatric Immunorheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, MIlan, Italy, ¹²Department of Medical of Health Sciences, Magna Graecia University, Catanzaro, Italy, ¹³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ¹⁴Institute for Maternal and Child Health, RCCS “Burlo Garofolo", Trieste, Italy, ¹⁵Department of Life Sciences and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy, ¹⁶Pediatric Rheumatology, Section Pediatric Service, Parc Taulí Sabadell University Hospital, Institute for Research and Innovation I3PT, Sant Joan de Deu Hospital, Autonomous University of Barcelona, Barcelona, Spain, ¹⁷Pediatric Rheumatology Unit, 1st Department of Pediatrics, Children's Hospital "Aghia Sophia", University of Athens, Athens, Greece, ¹⁸UOC Reumatologia e Malattie Autoinfiammatorie, Genoa, Italy, ¹⁹Rheumatology Unit, ERN ReConnet Center, Meyer Children's Hospital IRCCS, Florence Italy. NeuroFARBA Department, University of Florence, Florence, Italy

Meeting: ACR Convergence 2023

Keywords: Behçet's Syndrome, Vasculitis

Session Information

Date: Monday, November 13, 2023

Title: (1221–1255) Pediatric Rheumatology – Clinical Poster II: Connective Tissue Disease

Session Type: Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Arterial and venous thrombosis occurs in 6.6 to 38.4% of pediatric Behçet disease (BD) and cerebral sinus is one of the most involved districts. The aim of our study was to report clinical features and outcomes of a pediatric BD multicentre cohort with thrombosis, to identify possible predictive factors of thrombosis

Methods: A retrospective data collection of pediatric BD patients with thrombosis (T+) included in the EUROFEVER registry was conducted. Clinical data of BD patients without thrombosis (T-), belonging to the same contributing rheumatology units have been retrieved from EUROFEVER registry. T+ and T- groups were matched in a 2:1 ratio.

Results: 37 T+ patients were compared to 74 T- patients across 13 European pediatric rheumatology centers. BD onset occurred at a median age of 153 months (IQR±57) and 156 months (IQR±51) in the T+ and T- group, respectively. At onset, ICBD criteria fulfillment was significantly higher in the T- group compared to the T+ (p=0.015), whereas no differences were detected in ISG and PEDBD criteria frequencies. No gender differences were observed, Caucasian ethnicity significantly recurred in T- group while Middle Eastern in T+ group (p=0.002). HLA-B51 haplotype positivity was significantly reported in T- patients (p=0.04). At onset, pustulosis was most frequently observed in the T- group (p< 0.001) as well as gastrointestinal symptoms (p< 0.001) and ocular involvement (p=0.022). Conversely, neurological symptoms were more often described in T+ patients (p=0.034). As for T+ group, thrombosis was reported at BD presentation in 8/37 patients (29.6%). For the remaining patients, who developed thrombosis later in the disease course, the comparison of symptoms revealed that oral aphtosis (p=0.003), genital aphtosis (p=0.014) and posterior uveitis (p=0.050) were more frequently observed at BD onset than at thrombosis presentation, while pustulosis (p=0.02) and fever (p=0.019) significantly coexisted with thrombosis. Thrombosis type was mainly venous (26/37,70.3%), predominantly involving the cerebral sinuses (21/37, 56.8%). 16/29 (55.2%) T+ patients were on treatment before thrombosis onset with at least one systemic, non-steroid treatment. After thrombosis occurence, 35/37 (94.6%) T+ patients underwent or added an immunomodulatory treatment. 26/32 (81.3%) and 26/33 (21.2%) started anticoagulant and antiplatelet therapy, respectively. At a median follow up of 4 months (IQR±10), 9/28 (32.1%) T+ patients resolved thrombosis, 8/28 (28.6%) had a partial regression and 11/28 (39.3%) a persistence. A recurrence was reported in 6/31(19.4%) as venous thrombosis.

Conclusion: Middle Eastern ethnicity significantly recurred in T+ group, pustolosis and fever were more frequently concomitant to thrombosis. Neurological symptoms at onset could be related to thrombosis development in the BD course. Sinus veins were confirmed as the most frequent thrombosis site. Larger studies are required to better define predictive risk factors of thrombosis in pediatric BD.

Disclosures: M. Mastrolia: None; C. Matucci-Cerinic: None; S. Ozen: None; O. Kasapcopur: Novartis, 6, Pfizer, 6; C. Gaggiano: None; I. Kone-Paut: None; L. Cantarini: None; P. Dusser: None; Ü. Kaya-Akça: None; M. Yildiz: None; J. Brunner: None; G. Filocamo: None; R. Gallizzi: Novartis, 5, SOBI, 5; A. Insalaco: None; S. Pastore: None; D. Rigante: None; J. Sanchez-Manubens: None; E. Tsitsami: None; M. Gattorno: Novartis, 5, 6, Sobi, 5, 6; G. Simonini: Novartis, 5, SOBI, 5.

To cite this abstract in AMA style:

Mastrolia M, Matucci-Cerinic C, Ozen S, Kasapcopur O, Gaggiano C, Kone-Paut I, Cantarini L, Dusser P, Kaya-Akça Ü, Yildiz M, Brunner J, Filocamo G, Gallizzi R, Insalaco A, Pastore S, Rigante D, Sanchez-Manubens J, Tsitsami E, Gattorno M, Simonini G. Thrombotic Manifestations in Pediatric Behçet Disease Patients: A Multicentre Comparative Study from EUROFEVER Registry [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/thrombotic-manifestations-in-pediatric-behcet-disease-patients-a-multicentre-comparative-study-from-eurofever-registry/. Accessed .

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