Pediatric onset Behçet disease.

PURPOSE OF REVIEW: Behçet disease is becoming increasingly recognized in the pediatric rheumatology practice. Behçet disease remains a challenging vasculitis owing to the heterogeneity of clinical presentation, the disease course characterized by remissions and relapses and the lack of evidence-based data for its treatment. We need to understand the scope of the disease, its pathogenesis and the available treatment strategies for better management of these patients.

RECENT FINDINGS: Recent studies have shown that Behçet disease is much more frequent than we had thought. Genetic factors have long been implicated in the disease. Associations with candidate genes continue; however, the genome-wide association studies will probably provide us with more substantial data. This year, studies in childhood Behçet disease have been mainly gathered around eye diseases. Recommendations in adult Behçet disease for treatment strategies in eye diseases as well as different organ involvements will be reviewed along with the limited pediatric reports addressing treatment.

SUMMARY: The presented epidemiological data and specific clinical features of Behçet disease will enable us to understand the extent of the disease. It is important to understand the differences we encounter in children. Genetic associations may shed light on the pathogenesis and possibly treatment of Behçet disease. Since we lack sufficient pediatric data, we need to apply adult treatment strategies.