To assess the first-year features of patients with chronic nonbacterial osteomyelitis (CNO). Patients with a diagnosis of CNO, disease duration of under 13 months, and first registration in the German National Pediatric Rheumatologic Database (NPRD) between 2009 and 2018 were included in this cross-sectional analysis. Of 774 documented patients, 62.8% were female, and all patients had a median age of 11 years. The most affected clinical sites were the tibia (29.7%), pelvis (28.0%), and femur (27.8%). HLA-B27 was positive in 48 of 314 analyzed patients (15.3%). In 406 patients, an X-ray was performed at the first visit; X-ray results showed osteosclerosis/−lysis in 34.0% and hyperostosis in 14.5% of the patients. MRI scans (focal and whole-body scans) were performed in 648 patients, and 81.5% showed a positive TIRM/STIR signal. A total of 84.7% of the patients were administered nonsteroidal anti-inflammatory drugs, 9.6% were administered oral glucocorticoids, 10.8% were administered disease-modifying anti-rheumatic drugs (DMARDs), and 6.1% were administered bisphosphonates. An evaluation of the patient’s questionnaire showed an overall well-being (NRS 0–10) of 2.0. The PedCNO disease “activity” score revealed a 70% improvement in variables in 43% of patients in the initial 1-year follow-up. Copresentation with diagnostic criteria of pediatric enthesitis-related arthritis was rare. To our knowledge, the NPRD cohort seemed to be the largest cohort of children and adolescents suffering from CNO worldwide. Most patients were treated effectively with NSAIDs, and only a small group of patients was administered additional medication. The patient-defined measures of disease activity had a moderate impact on patients’ daily lives. Not applicable.

中文翻译：

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儿童慢性非细菌性骨髓炎第一年随访——2009-2018年德国国家小儿风湿病数据库分析

评估慢性非细菌性骨髓炎 (CNO) 患者的第一年特征。2009 年至 2018 年间诊断为 CNO、病程小于 13 个月且首次在德国国家儿科风湿病数据库 (NPRD) 登记的患者被纳入该横断面分析。在 774 名记录在案的患者中，62.8% 为女性，所有患者的中位年龄为 11 岁。受影响最严重的临床部位是胫骨 (29.7%)、骨盆 (28.0%) 和股骨 (27.8%)。HLA-B27 在 314 名分析患者中的 48 名 (15.3%) 呈阳性。406 名患者在第一次就诊时进行了 X 光检查；X 射线结果显示 34.0% 的患者出现骨硬化/-溶解，14.5% 的患者出现骨质增生。对 648 名患者进行了 MRI 扫描（局部和全身扫描），81.5% 的患者显示出阳性 TIRM/STIR 信号。共 84 个。7% 的患者服用非甾体抗炎药，9.6% 服用口服糖皮质激素，10.8% 服用缓解疾病的抗风湿药 (DMARDs)，6.1% 服用双膦酸盐。对患者问卷的评估显示整体幸福感 (NRS 0-10) 为 2.0。PedCNO 疾病“活动”评分显示，在最初的 1 年随访中，43% 的患者的变量改善了 70%。与小儿附着点炎相关关节炎的诊断标准同时出现的情况很少见。据我们所知，NPRD 队列似乎是全球最大的患有 CNO 的儿童和青少年队列。大多数患者使用非甾体抗炎药进行了有效治疗，只有一小部分患者接受了额外的药物治疗。患者定义的疾病活动量度对患者的日常生活有中等影响。不适用。