Clinical course of pediatric-onset Behçet's Disease in young adulthood

Abstract

Objectives Although Behçet's disease (BD) typically manifests in the second or third decade of life, initial symptoms may appear at a younger age. It may also take a longer time for the full disease phenotype to develop after the first symptom onset in pediatric patients. In this study we aimed to assess the clinical course of pediatric-onset BD in adulthood period. Methods The files of 112 patients diagnosed with BD before the age of 18, selected from five tertiary clinics, were retrospectively examined. Patients with a follow-up of less than six months were excluded. Results The study comprised 93 patients with pediatric-onset BD, of whom 64.5% (n = 60) were male. The median age of diagnosis was 15 years (13–17). Major organ involvement was present in 49 (52.5%) patients. The most commonly affected organ was the eye (29%). Sixty-eight (73.1%) patients had follow-up data in adulthood. Fourty patients had only mucocutaneous manifestations in the pediatric period. During follow-up in adulthood, 15 (53.3% were male) had new major organ involvement with a mean of 10.1 (SD: 7.9) years after diagnosis. Twenty-eight patients (41.1%) experienced major organ involvement during the pediatric period. In adulthood follow-up, 12 (42.8%) developed new major organ involvement and/or relapse of the same organ. Eighteen (26.5%) of 68 pediatric-onset BD patients had new major organ involvement, and 9 (13.2%) had a relapse during adulthood follow-up. Conclusion Our results showed that nearly one-third of pediatric BD patients have a new major organ involvement or a relapse in adulthood. Regular follow-up of pediatric BD patients in adulthood is essential to prevent long-term damage in this disease subset.