OBJECTIVE: 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.
CONCLUSIONS: 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.
目的:为了评估人口统计学,病因学,并发症,治疗,小儿非感染性肉芽肿性葡萄膜炎的视觉预后。
方法:从儿科风湿病科招募的16岁前儿童非感染性PGU的回顾性图表回顾,Bicettre医院,法国,从2001年到2023年。
结果:我们包括50例患者,其中90只受影响的眼睛:29例特发性葡萄膜炎,15患有结节病,5患有幼年特发性关节炎,还有一个患有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%),双边(84%),慢性(84%)。结节病是特发性疾病后最常见的诊断,特别是在存在淋巴细胞减少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眼中有68眼(76%)出现眼部并发症,尤其是在全葡萄膜炎病例中。最常用的治疗是全身性皮质类固醇(72%)和甲氨蝶呤(80%)。在最后一次随访时,23%的眼睛处于缓解状态,68%无活性,4%保持活性。中位随访时间为5.8年。
结论:我们报告了PGU的最大队列。PGU多为特发性,并发症发生率高。结节病和特发性全葡萄膜炎是严重的疾病,在诊断时应开始疾病修饰治疗以改善管理。