P051 Juvenile Behcet's disease

Abstract

Abstract Background Behçet's disease is a rare systemic vasculitis in children. The most frequent manifestations are orogenitalaphthosis and uveitis. Neurological, digestive, vascular, joint, and skin involvement are possible. We aimed to describe the clinical, diagnostic criteria, and therapeutic features of pediatric-onset Behcet's disease Methods Study carried out based on files collected over 12 years (2007–2018) concerning patients under the age of 15 hospitalized in the pediatric department of CHU de Blida for Behcet disease Results For 12 years (2007–2018), we hospitalized five children for various reasons and whose diagnosis of Behcet's disease was retained. These are 03 boys (2 of the theme are brothers) and 02 girls. The average age at admission was 13.5 years (12–15 years), and the average duration of follow-up is 4.2 years (15 months-8 years). The mean age of onset of first symptoms was 11 years, and the meantime to diagnosis was 2 years (12 months-3 years) The main diagnostic criteria were: mouth ulcers in five patients (100%), genital ulcers in three patients (60%), ophthalmic manifestations in two patients (40%): one had bilateral uveitis, and the other had papillitis, the pathergy test was positive in 3 patients (60%). The other manifestations were as follows: polyarthralgia in 3 patients (60%), family history in one patient (20%). Neurological manifestations were present in 3 patients (60%): one child presented two episodes of aseptic meningitis and thrombosis deep cerebral venous; one child with diffuse cerebral demyelination lesions, and the third with HIC syndrome and massive lateral sinus thrombosis. Three children have a vascular involvement (60%): pulmonary embolism with thrombosis of the superior vena cava (20%), bilateral thrombosis of the jugular veins (20%), and thrombosis of the right ventricle complicated by pulmonary embolism (20%) Initial symptoms included recurrent aphthous stomatitis (100%), arthralgia (60%), prolonged fever (20%), behavioral disturbances and dysarthria (20%), weight loss (40%), and headache (60 %). The histocompatibility antigen (HLAB 51) was found in 2 children (40%). The treatment included corticosteroids for all patients (3 boluses of 1 g/m2/day of methylprednisolone then relayed by prednisone 1 mg/kg/day), azathioprine 2.5 mg/kg/day in 5 children (100%), and colchicine for 2 patients only one patient had received anticoagulant therapy (LMWH followed by VKA) Relapses were observed in a single patient (20%) and mortality in one patient (20%.) Conclusion Behcet's disease is a rare disease whose diagnosis remains difficult in children. However, early diagnosis and aggressive treatment are mandatory to avoid complications and functional sequelae.