Minor salivary gland biopsy in the diagnosis of definite ocular sarcoidosis in paediatric granulomatous uveitis.

IF 2.8 3区医学 Q1 PEDIATRICS

Pediatric Rheumatology Pub Date : 2025-03-10 DOI:10.1186/s12969-025-01078-3

Leire Etchandy, Marie-Noelle Meaux, Olivier Richer, Chloé Bianchi, Skander Korbi, Claire Castain, Guillaume Chotard, Marie-Bénédicte Rougier, Emmanuel Ribeiro, Johanna Clet, Pascal Pillet, Jérôme Granel

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引用次数: 0

Abstract

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.

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小唾液腺活检在小儿肉芽肿性葡萄膜炎明确眼结节病诊断中的价值。

背景：非传染性儿童肉芽肿性葡萄膜炎（PGU）是一种罕见的疾病，在一半以上的患病儿童中是特发性的。明确的眼部结节病（OS）的诊断必须得到活检中检测到的非干酪化肉芽肿的支持，因此对PGU患儿来说是一个挑战。本研究探讨了小涎腺活检（MSGB）在PGU明确OS诊断中的应用。方法：纳入26例2018年至2023年间诊断为PGU的连续儿童，并在诊断后3个月内系统地进行了MSGB。结果：PGU诊断的中位年龄为11.6（4.2 ~ 16.5）岁，男孩占54%。PGU主要包括双侧（92%）和泛葡萄膜炎（96%）。在12/26（46%）的儿童中，MSGB检测到非干酪化肉芽肿（MSGB+）。总的来说，26名儿童中有13名（50%）被诊断为明确的OS， 8名（31%）患有特发性葡萄膜炎。MSGB诊断PGU患儿明确OS的敏感性为92%，NPV为93%。与MSGB-儿童相比，MSGB +儿童在诊断时往往大于10岁（p = 0.02），一般体征（p = 0.003），眼外器官受损伤（p = 0.005）和多克隆高γ -球蛋白血症（p = 0.03）的发生率更高。在OS诊断中，最常见的眼外器官受累是肾脏（46%）和胸部（46%）。88%的儿童的一线治疗是全身性皮质类固醇。在PGU诊断后的中位随访时间为3.1（0.6-6.3）年，88%的儿童需要甲氨蝶呤和/或抗肿瘤坏死因子- α治疗以达到非活动性葡萄膜炎。结论：MSGB有助于提高OS的诊断，减少PGU中特发性葡萄膜炎的发生率。PGU患者可以考虑MSGB，特别是那些年龄在10 ~ 10岁，有一般体征和/或高γ -球蛋白血症的患者。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY

CiteScore

4.10

自引率

8.00%

发文量

审稿时长

>12 weeks

期刊介绍： Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.