Paediatric-Onset Behçet’s Disease Often Worsens in Adulthood - EMJ

Paediatric-Onset Behçet’s Disease Often Worsens in Adulthood

A NEW study reveals that nearly one-third of paediatric-onset Behçet’s disease (BD) patients experience new major organ involvement or disease relapse in adulthood, underscoring the importance of long-term monitoring. The findings highlight the progressive nature of BD, a rare inflammatory condition that can initially manifest in childhood but worsen with age. 

The study retrospectively examined 93 patients diagnosed with BD before the age of 18 at five tertiary clinics, with follow-up data available for 68 patients into adulthood. The median age of diagnosis was 15 years, with 64.5% of the cohort being male. During the paediatric period, 40 patients exhibited only mucocutaneous symptoms, while 28 (41.1%) experienced major organ involvement, most commonly affecting the eyes (29%). 

In adulthood, new complications arose for 18 of the 68 patients (26.5%), and nine patients (13.2%) experienced relapses of previously affected organs. Among those with initially limited symptoms, 15 (53.3% male) developed major organ involvement an average of 10.1 years after diagnosis. For those with organ involvement in childhood, 12 patients (42.8%) experienced new complications or relapses in adulthood. 

The results emphasise the chronic and unpredictable course of paediatric-onset BD, which requires vigilant follow-up well into adulthood to manage disease progression and minimise long-term damage. 

Reference 

Bozkurt T et al. Clinical course of pediatric-onset Behçet’s Disease in young adulthood. Rheumatology. 2024;DOI: 10.1093/rheumatology/keae624. 

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