Age-related differences in the clinical phenotypes of Behçet’s disease: The experience of two referral centres

Abstract

ABSTRACT Objectives Behçet’s disease (BD) is a multisystem disease and frequently occurs during the second–fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD. Materials and methods This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13–17 years), adult onset (18–39 years), and late onset (>40 years). Results The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group. Conclusion There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic.