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.

中文翻译：

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儿童早期发病的白塞病的临床病程


目的 虽然白塞病 （BD） 通常在生命的第二个或第三个十年出现，但初始症状可能在年轻时出现。在儿科患者首次出现症状后，完整的疾病表型也可能需要更长的时间才能发展。在这项研究中，我们旨在评估成年期儿童发病 BD 的临床病程。方法 回顾性检查从 5 家三级诊所选育的 112 例 18 岁前诊断为 BD 的患者的档案。随访时间少于 6 个月的患者被排除在外。结果 该研究包括 93 例儿童发病的 BD 患者，其中 64.5% （n = 60） 为男性。中位诊断年龄为 15 岁 （13-17）。49 例 （52.5%） 患者存在主要器官受累。最常受影响的器官是眼睛 （29%）。68 例 （73.1%） 患者在成年后有随访数据。40 例患者在儿科期间仅出现皮肤黏膜表现。在成年期随访期间，15 例 （53.3% 为男性） 在诊断后平均 10.1 （SD： 7.9） 年出现新的主要器官受累。28 例患者 （41.1%） 在儿科期间经历了主要器官受累。在成年随访中，12 例 （42.8%） 出现新的主要器官受累和/或同一器官复发。68 例儿科发病的 BD 患者中有 18 例 （26.5%） 有新的主要器官受累，9 例 （13.2%） 在成年随访期间复发。结论 我们的结果显示，近 1/3 的儿科 BD 患者在成年后出现新发的主要器官受累或复发。成年后对儿科 BD 患者进行定期随访对于防止该疾病亚群的长期损害至关重要。