Childhood Behçet's disease: clinical features and comparison with adult-onset disease

To study the clinical spectrum of Behçet's disease (BD) in childhood, in comparison to adult-onset disease.

Nineteen children, who fulfilled disease criteria up to the age of 16 yr, were studied. The results were compared to those of 34 adult patients with BD. An activity index and severity score were calculated for both study groups.

The mean age of disease onset was 6.9+/-3.9 yr, similar ages of onset were found in males and females. The clinical spectrum of childhood BD resembled that of adult disease; however, the prevalence of certain manifestations was different between children and adults. Children with BD had significantly less genital ulcers, less vascular thromboses and more non-specific gastrointestinal symptoms, as well as central nervous system involvement and arthralgia. A relatively high prevalence of uveitis was found in childhood BD. The activity index and severity score were significantly lower in children than in adults.

Our results point to a similar systemic expression of BD in children and adults; however, the disease seems to run a less severe course in children.