Abstract

Aim: There is little information on the demographic and clinical characteristics of Behçet’s disease in children in different parts of the world. We sought to provide this information through a questionnaire survey of specialist eye centres.

Methods: Descriptive questionnaires were collected from 25 eye centres in 14 countries. The questionnaire surveyed details of juvenile-onset Behçet’s disease with uveitis. Ethnic groups, clinical features, treatments and prognosis of paediatric-age Behçet’s disease were examined on a worldwide scale.

Results: The clinical data of 135 juvenile-onset and 1227 adult-onset patients with uveitis were collected. The average age of disease diagnosis in the children was 11.7 years old. Of the ethnic groups identified 54% were from Middle East, 43% from Europe, but only 2% from East/South Asian countries. By contrast, 19.2% of adult patients were from East or South Asia. The frequency of genital ulcers in juvenile patients was 38.7%, which was significantly lower than in adult cases (53.5%; p<0.01).

Conclusions: Behçet’s disease with uveitis was less common in children than in adults in East/South Asia. Although the clinical features of the systemic disease were similar in children and adults, there was a lower frequency of genital ulceration in children.