小唾液腺活检在小儿肉芽肿性葡萄膜炎确诊眼部结节病中的应用
Minor salivary gland biopsy in the diagnosis of definite ocular sarcoidosis in paediatric granulomatous uveitis.
作者信息
Etchandy Leire, Meaux Marie-Noelle, Richer Olivier, Bianchi Chloé, Korbi Skander, Castain Claire, Chotard Guillaume, Rougier Marie-Bénédicte, Ribeiro Emmanuel, Clet Johanna, Pillet Pascal, Granel Jérôme
机构信息
Paediatric Department, Côte Basque Hospital Centre, Bayonne, France.
Paediatric Clinical Immunology, Pellegrin Hospital, INSERM CICP, University Hospital Centre of Bordeaux, Bordeaux, CIC1401, France.
出版信息
Pediatr Rheumatol Online J. 2025 Mar 10;23(1):23. doi: 10.1186/s12969-025-01078-3.
BACKGROUND
Non-infectious paediatric granulomatous uveitis (PGU) is a rare disease that is idiopathic in more than half of affected children. The diagnosis of definite ocular sarcoidosis (OS) must be supported by the presence of non-caseating granulomas detected in biopsy, and is therefore a challenge in children with PGU. This study investigated the utility of minor salivary gland biopsy (MSGB) in the diagnosis of definite OS in PGU.
METHODS
Twenty-six consecutive children with PGU diagnosed between 2018 and 2023 and with a systematically performed MSGB within 3 months of the diagnosis were enrolled.
RESULTS
The median age at PGU diagnosis was 11.6 (4.2-16.5) years, and 54% of the children were boys. PGU consisted mainly of bilateral (92%) pan-uveitis (96%). MSGB detected non-caseating granulomas (MSGB+) in 12/26 (46%) children. In all, 13 of the 26 (50%) children were diagnosed with definite OS, and 8 (31%) had idiopathic uveitis. MSGB had a sensitivity of 92%, and a NPV of 93% in the diagnosis of definite OS in children with PGU. Compared to MSGB- children, those who were MSGB + were more frequently older than 10 years of age at diagnosis (p = 0.02), had a higher rate of general signs (p = 0.003), extra-ocular organ involvement (p = 0.005) and polyclonal hypergammaglobulinaemia (p = 0.03). The most frequent extra-ocular organ involvements at OS diagnosis were renal (46%) and thoracic (46%). First-line therapy was systemic corticosteroids in 88% of the children. During a median follow-up time of 3.1 (0.6-6.3) years after PGU diagnosis, 88% of the children needed methotrexate and/or anti-tumour necrosis factor-alpha therapy to achieve inactive uveitis.
CONCLUSIONS
MSGB is useful to improve the diagnosis of OS and to reduce the incidence of uveitis considered idiopathic in PGU. MSGB could be considered in PGU patients, particularly those > 10 years of age with general signs and/or hypergammaglobulinaemia.
背景
非感染性儿童肉芽肿性葡萄膜炎（PGU）是一种罕见疾病，半数以上患病儿童病因不明。确诊眼结节病（OS）必须有活检发现的非干酪样肉芽肿支持，因此对于PGU患儿来说是一项挑战。本研究探讨了小唾液腺活检（MSGB）在PGU确诊OS中的应用价值。
方法
纳入2018年至2023年间连续诊断为PGU且在诊断后3个月内系统进行了MSGB的26例儿童。
结果
PGU确诊时的中位年龄为11.6（4.2 - 16.5）岁，54%的儿童为男孩。PGU主要表现为双侧（92%）全葡萄膜炎（96%）。MSGB在12/26（46%）的儿童中检测到非干酪样肉芽肿（MSGB+）。26例儿童中共有13例（50%）被诊断为确诊OS，8例（31%）患有特发性葡萄膜炎。MSGB在PGU患儿确诊OS中的敏感性为92%，阴性预测值为93%。与MSGB-的儿童相比，MSGB+的儿童在诊断时年龄大于10岁的频率更高（p = 0.02），全身症状发生率更高（p = 0.003），眼外器官受累率更高（p = 0.005）以及多克隆高球蛋白血症发生率更高（p = 0.03）。OS诊断时最常见的眼外器官受累是肾脏（46%）和胸部（46%）。88%的儿童一线治疗为全身使用糖皮质激素。在PGU诊断后的中位随访时间3.1（0.6 - 6.3）年期间，88%的儿童需要甲氨蝶呤和/或抗肿瘤坏死因子-α治疗以实现葡萄膜炎静止。
结论
MSGB有助于提高OS的诊断，并降低PGU中被认为是特发性葡萄膜炎的发生率。PGU患者，特别是年龄大于10岁且有全身症状和/或高球蛋白血症的患者，可考虑进行MSGB。
Zotero 插件