Unal Dilara, Yigit Yasemin Demir, Basaran Ozge, Yildiz Adalet Elcin, Bayindir Yagmur, Cam Veysel, Emreol Hülya Ercan, Erkan Mehmet Orhan, Banak Ozlem Necipoglu, Sag Erdal, Bilginer Yelda, Aydingoz Ustun, Ozen Seza
Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey.
Division of Pediatric Rheumatology, Faculty of Medicine, Fırat University, Elazig, Turkey.
Clin Rheumatol. 2025 Aug 26. doi: 10.1007/s10067-025-07653-z.
Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disorder primarily affecting children and adolescents. An international consensus group developed the EULAR/ACR Classification Criteria in order to define a homogeneous group of patients diagnosed with CNO. This study aims to validate these criteria in a diverse pediatric cohort and compare their performance with the Jansson and Roderick diagnostic criteria.
A retrospective evaluation was conducted on 78 pediatric CNO patients and 99 mimickers in Hacettepe University department of pediatric Rheumatology from 2020 to 2024. Demographic, clinical, laboratory, and imaging data were analyzed, and the sensitivity, specificity, and predictive values of the EULAR/ACR criteria were assessed. Statistical comparisons were made against the Jansson and Roderick criteria.
The EULAR/ACR criteria demonstrated high accuracy, with a sensitivity of 92.68% and specificity of 97.89%, outperforming the Jansson criteria (sensitivity: 88.46%, specificity: 72.73%) and aligning closely with the Roderick criteria (sensitivity: 96.15%, specificity: 96.97%). Key distinguishing features included multifocal and symmetric lesions, frequent involvement of high-scoring anatomical sites (e.g., clavicle and mandible), and the absence of exclusion criteria such as fever and markedly elevated inflammatory markers.
The newly validated EULAR/ACR classification criteria for pediatric CNO demonstrated high specificity and good sensitivity in our cohort. Their application facilitates the identification of homogeneous patient populations, aiding in research consistency and the development of standardized approaches to CNO classification and management. Key Points • The EULAR/ACR classification criteria for pediatric CNO exhibited high specificity (97.89%) and sensitivity (92.68%), supporting their validity. • Multifocal and symmetric bone lesions, particularly in the clavicle and mandible, strongly support classification under these criteria. • Validation in a pediatric cohort reinforces their utility for standardized patient classification and research consistency.
慢性非细菌性骨髓炎(CNO)是一种罕见的自身炎症性骨病,主要影响儿童和青少年。一个国际共识小组制定了欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)分类标准,以界定诊断为CNO的一组同质患者。本研究旨在在一个多样化的儿科队列中验证这些标准,并将其性能与扬松(Jansson)和罗德里克(Roderick)诊断标准进行比较。
对2020年至2024年在哈杰泰佩大学儿科风湿病科的78例儿科CNO患者和99例模仿者进行回顾性评估。分析了人口统计学、临床、实验室和影像学数据,并评估了EULAR/ACR标准的敏感性、特异性和预测值。与扬松和罗德里克标准进行了统计学比较。
EULAR/ACR标准显示出高准确性,敏感性为92.68%,特异性为97.89%,优于扬松标准(敏感性:88.46%,特异性:72.73%),并与罗德里克标准密切一致(敏感性:96.15%,特异性:96.97%)。关键的鉴别特征包括多灶性和对称性病变、高分解剖部位(如锁骨和下颌骨)的频繁受累,以及不存在发热和炎症标志物明显升高等排除标准。
新验证的儿科CNO的EULAR/ACR分类标准在我们的队列中显示出高特异性和良好的敏感性。它们的应用有助于识别同质的患者群体,有助于研究的一致性以及CNO分类和管理标准化方法的发展。要点 • 儿科CNO的EULAR/ACR分类标准表现出高特异性(97.89%)和敏感性(92.68%),支持其有效性。 • 多灶性和对称性骨病变,特别是在锁骨和下颌骨,强烈支持根据这些标准进行分类。 • 在儿科队列中的验证加强了它们在标准化患者分类和研究一致性方面的效用。