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.
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Funding This study was supported by grants from the Ministry of Education, Culture, Sports, Science and Technology (MEXT) Japan, and the Ministry of Health, Labor and Welfare Japan.
Competing interests None declared.
Provenance and Peer review Not commissioned; externally peer reviewed.
Ethics approval Ethics approval was obtained from the Institutional Review Board of Hokkaido University Hospital for Clinical Research (#009-0065).
Patient consent Obtained.