RESUMO
OBJECTIVES: Behçet's syndrome (BS) is a rare multi-system inflammatory disorder. Clinical phenotypic variance across geographical regions is recognised but UK BS patients' variance by age groups and gender has not been studied. This study compares the clinical features of adult and juvenile onset Behçet's Syndrome (JBS) in a UK population. METHODS: Two clinical databases of BS patients were compared. The JBS database was collected at the Great Ormond Street Hospital for Children, London (n=46). The adult database was collected at the Hammersmith Hospital, London (n=560). RESULTS: Oro-genital aphthosis had high prevalence in both the JBS and the adult cohort (oral: 97.8% vs. 96.6%, genital: 73.9% vs. 75.7%). The JBS cohort was more likely to have gastrointestinal involvement (21.7% vs. 4.5%, p<0.001) and arthritis (21.7% vs. 9.6%, p=0.021) compared to adults. The JBS cohort was less likely to have eye involvement (4.3% vs. 37%, p<0.001), skin (21.7% vs. 55.4%, p<0.001) and vascular involvement (6.5% vs. 17.5% p=0.063). JBS females had a higher rate of genital aphthosis than JBS males (87.5% vs. 59.1%, p=0.044). Adult females had higher rates of genital (85.2% vs. 64.5%, p<0.001) and oral (99.0% vs. 93.8%, p=0.001) aphthosis than adult males. Adult males were more likely to have ophthalmological (44.9% vs. 30.3%, p<0.001) and vascular (23.0% vs. 12.8%, p=0.002) manifestations than adult females. CONCLUSIONS: UK JBS patients displayed less ocular and skin manifestations compared to the adult BS patients. This information will aid clinicians in diagnosing BS in UK adult and paediatric populations.
