Background: Behçet’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 Behçet’s Disease patients with cerebral venous sinus thrombosis (CVST) and to review previous studies reporting the clinical characteristics and outcomes of Juvenile Behçet’s Disease with CVST.

Methods: The patients who met Pediatric Behçet’s Disease (PEDBD) classification criteria for juvenile Behçet’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., über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre 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 Acarı: None declared, özge Basaran: None declared, Erdal Sag: None declared, Kader Karlı Oğuz: None declared, Yelda Bilginer: None declared, Erbil Unsal Grant/research support from: Novartis, AbbVie, Roche, Koçak Pharma, Speakers bureau: Novartis, AbbVie, Roche, Koçak Pharma, Seza özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche

DOI: 10.1136/annrheumdis-2019-eular.4574