Pediatric-onset Behçet disease

Behçet disease has recently been classified as a variable vessel vasculitis. This disease is variable not only in the vessel type it selects, but also in its clinical presentation. In fact, the heterogeneity of the disease has been a drawback in understanding the etiopathogenesis of the disease. This review will address the recent developments in our understanding of the genetic background and pathogenesis of Behçet disease, as well as the analysis of clinical features.

Recent genome-wide association studies mainly confirm the association with HLA-B51 and highlight the association with IL23/IL17 pathway and IL10, and a molecule that functions in the loading of peptides to HLA Class I molecules. Immunological studies also support the role of IL17 in the disease pathogenesis. Clinical studies in Behçet disease have provided us with clearer definitions of the vascular and central nervous system involvement in Behçet disease. An international effort to delineate the characteristics of pediatric patients has shown us that we need pediatric classification criteria in children with higher sensitivity. As to the treatment of the disease, new biological drugs seem to offer promising results in resistant cases.

The new pathways defined in the disease will not only help us better understand the pathogenesis, but also help us in more targeted therapy. Although pediatric cases are being increasingly recognized, the heterogeneity of the disease presents an obstacle for studies. Thus, we can reach conclusive results with multicenter studies only.