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Maladie de Behçet pédiatrique : une entité à évoquer en cas d’uvéite sévère…

Behçet’s disease (BD) is an inflammatory and multisystemic condition involving cutaneous, ocular and vascular manifestations. Ocular findings comprise anterior and posterior uveitis associated with retinal vasculitis. BD in children is rare and challenging to recognize as clinical picture may be incomplete. In this study, we aimed to report cases of uveitis in pediatric patients with BD and provide a review of the literature.

In this case series we included pediatric patients with BD diagnosed and managed in the Departments of Ophthalmology and Pediatric Rheumatology of, Bicêtre-Paris Saclay hospital between 2012 and 2020. We retrospectively collected demographic, ophthalmological and systemic data at presentation and during follow-up. 

Four patients, aged 13±2.9 years (9-16) were included. They were all from North African descent.  One patient was heterozygote for familial Mediterranean fever. Oral aphtosis was present in 3 cases, none of them had bipolar aphtosis. Neurological manifestations were present in 3 cases. Ocular involvement led to the diagnosis of BD in all of our cases. Three patients met the consensus diagnostic criteria for pediatric BD, while 1 patient with typical neurological and ocular involvement was diagnosed as BD after multidisciplinary agreement. Presenting ocular symptoms were red eye (2/4) and loss of vision (4/4). Posterior uveitis with retinal vasculitis, papillitis and macular edema was present in all patients, with associated anterior uveitis in 2 cases. Other features included occlusive vasculitis (2/4), necrotizing retinitis (2/4). Corticosteroid combined with azathioprine and anti-TNFα were administered in all patients. One case also received ciclophosphamide pulses. One patient with severe optic neuropathy had complete bilateral vision loss caused by optic atrophy during follow-up. Among the 3 remaining, visual acuity of the most severe eye improved from 1.5±1.1 (0.2-2.3) at presentation to 0.0±0.1LogMAR (0-0.1) at last visit. No patient relapsed during follow-up. 

Our case series suggest that ocular manifestations of pediatric BD are comparable to those seen in adult patients and may be particularly severe. 

Pediatric BD is a rare but potentially blinding disease that must be suspected in cases of compatible clinical pictures. As in adult patients, aggressive and long-term treatment is mandatory to prevent vision loss and recurrences.