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• W2949830018 abstract "Background Behcet’s disease (BD) is an autoinflammatory disorder which may present with multi-systemic involvements including the vascular, cutaneous, articular, gastrointestinal, and/or central nervous systems (CNS). CNS involvement appears in two main groups: parenchymal or non-parenchymal. Neurological symptoms in children and adolescents can be confused with many other disorders and may be the initial symptom of BD. Objectives To report our experiences of the juvenile Behcet’s Disease patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of Juvenile Behcet’s Disease with CVST. Methods The patients who met Pediatric Behcet’s Disease (PEDBD) classification criteria for juvenile Behcet’s Disease from 3 referral centers in Turkey were reviewed retrospectively. Disease activity was assessed by BD current activity form (BDCAF). A systematic review of literature of all published data was conducted. Results The study group consisted of 12 juvenile BD patients with CVST. At the time of CVST diagnosis, the most common symptom was headache (%100), followed by vomiting (25%), blurred vision (16.6-7%), and disturbances in eye movements (16.7%). Six (50%) patients presented with sinus venous thrombosis as an initial symptom. Transverse sinus was the most frequently affected sinus (9/12, 75%) followed by superior sagittal sinus (8/12, 66.6%) and sigmoid sinus (1/12, 8.3%). The median (minimum-maximum) BDCAF was 6 (5-8). Four children (33.3%) had another venous thrombosis apart from CVST. All patients received pulse methylprednisolone for three consecutive days continued with oral prednisolone. Steroid treatment was tapered and discontinued minimum in six months. Eleven patients received azathioprine concomitant to steroid treatment at the time of CVST. All the patients received anticoagulant therapy concomitantly. Only one patient had relapse. Median (min-max) follow-up period was 4 years (1-10). In the literature review, we identified nine articles, describing 35 pediatric CVST patients associated with BD. Thirty patients achieved remission, while five patients had residual neurologic deficit. Conclusion Further multicenter studies with more patients and prospective follow-up may help us to understand the whole spectrum in these patients. References [1] Behcet, H., uber rezidivierende, aphtose, durch ein Virus verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermatologische Wochenschrift, 1937(105): p.1152-63. [2] Metreau-Vastel, J., et al., Neurological involvement in paediatric Behcet’s disease. Neuropediatrics, 2010. 41(5): p.228-34. Disclosure of Interests Selcan Demir: None declared, Ceyhun Acari: None declared, ozge Basaran: None declared, Erdal Sag: None declared, Kader Karli Oguz: None declared, Yelda Bilginer: None declared, Erbil Unsal Grant/research support from: Novartis, AbbVie, Roche, Kocak Pharma, Speakers bureau: Novartis, AbbVie, Roche, Kocak Pharma, Seza ozen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche" @default.
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• W2949830018 date "2019-06-01" @default.
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• W2949830018 title "AB0958 PEDIATRIC BEHCET’S DISEASE WITH SINUS VENOUS THROMBOSIS: THREE CENTER EXPERIENCE FROM TURKEY" @default.
• W2949830018 doi "https://doi.org/10.1136/annrheumdis-2019-eular.4574" @default.
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