Introduction Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging. Objectives To evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis. Methods Retrospective chart review of non-infectious PGU occurring in children before the age of 16 years recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023. Results We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with juvenile idiopathic arthritis, and one with Vogt-Koyanagi-Harada disease. Median age at diagnosis was 9.8 years (range 7.2–12.5). The sex-ratio M/F was 0.52. The most common features of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%) particularly in cases of panuveitis. The most commonly used treatments were systemic corticosteroids (72%) and methotrexate (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years. Conclusion We report the largest cohort of PGU. PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management.

中文翻译：

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儿童非感染性肉芽肿性葡萄膜炎：一项回顾性队列研究

简介 小儿肉芽肿性葡萄膜炎 (PGU) 很罕见。此外，缺乏认识往往会导致诊断延迟和视力结果不佳。确定根本原因并决定如何最好地治疗每个患者是具有挑战性的。目的 评估儿童非感染性肉芽肿性葡萄膜炎的人口统计学、病因、并发症、治疗和视力预后。方法 对 2001 年至 2023 年从法国比塞特医院小儿风湿病科招募的 16 岁以下儿童中发生的非感染性 PGU 进行回顾性图表审查。 结果 我们纳入了 50 名患者，90 只受影响的眼睛：29 名患有特发性葡萄膜炎， 15 例患有结节病，5 例患有幼年特发性关节炎，1 例患有 Vogt-Koyanagi-Harada 病。诊断时的中位年龄为 9.8 岁（范围 7.2-12.5）。性别比M/F为0.52。 PGU 最常见的特征是：全葡萄膜炎（56%）、双侧葡萄膜炎（84%）和慢性（84%）。结节病是继特发性疾病之后最常见的诊断，特别是在存在淋巴细胞减少症和高丙种球蛋白血症的情况下。 12% 的病例存在葡萄膜脑膜炎。诊断后，90 只眼睛中有 68 只 (76%) 出现眼部并发症，尤其是全葡萄膜炎病例。最常用的治疗方法是全身性皮质类固醇（72%）和甲氨蝶呤（80%）。在最后一次随访中，23% 的眼睛处于缓解状态，68% 的眼睛不活动，4% 的眼睛保持活动状态。中位随访时间为 5.8 年。结论 我们报告了最大的 PGU 队列。 PGU 大多是特发性的，并发症发生率很高。结节病和特发性全葡萄膜炎是严重疾病，应在诊断时开始疾病缓解治疗以改善治疗。