Abstract：
BACKGROUND: 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.
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.
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.
摘要：
背景：小儿肉芽肿性葡萄膜炎（PGU）很少见。此外,缺乏意识通常会导致诊断延迟和视力不良。确定根本原因并决定如何最好地治疗每位患者具有挑战性。
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。
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。
