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[Behçet's disease: pediatric features]
1Service de Pédiatrie, Hôpital Nord, Marseille. ikonepau@ap-hm.fr
Insights
Pediatric Behçet's disease (BD) presents unique symptoms like fever and abdominal pain, with severe uveitis. Genetic factors likely influence early disease onset, necessitating clear distinctions between pediatric and juvenile BD groups.
Area of Science:
- Rheumatology
- Ophthalmology
- Genetics
Context:
- Growing recognition of pediatric Behçet's disease (BD) in global literature.
- Surveys reveal distinct clinical manifestations in children, including recurrent fever and abdominal pain.
- Childhood-onset uveitis in BD is notably severe.
Purpose:
- To highlight the unique characteristics of pediatric Behçet's disease.
- To emphasize the severe ocular manifestations (uveitis) in affected children.
- To discuss the significant role of genetic factors and anticipation in early disease onset.
Summary:
- Pediatric BD exhibits specific symptoms like fever and abdominal pain, differing from adult presentations.
- Uveitis in childhood BD is severe and requires specific attention.
- Genetic predisposition and anticipation are key factors in the early manifestation of pediatric BD.
- A critical need exists to differentiate between 'pediatric BD' (onset before 16) and 'juvenile BD' (adults with childhood onset).
Impact:
- Improved diagnostic criteria for pediatric Behçet's disease.
- Enhanced understanding of the genetic underpinnings of early-onset BD.
- Development of targeted therapeutic strategies for severe pediatric manifestations like uveitis.
- Clarification of classification for pediatric and juvenile BD cohorts for research and clinical practice.
Abstract:
There is an increased awareness about the pediatric aspects of Behçet's disease (BD) in the world literature. Recent surveys of patients have outlined peculiar features in this age group such as recurrent attacks of fever and abdominal pain. Considered to be uncommon in childhood, uveitis have a very severe course. The higher frequency of familial cases and the genetic anticipation suggest that the genetic component plays a major role in the early expression of the disease. Pediatric aspects of BD including different categories of patients, are not definitely characterized. Indeed it is now necessary to distinguish the pediatric group (BD completed before 16 years) from the "juvenile" group (adult patients with first symptoms before 16 years).