Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Department of rheumatology, Children's Hospital of Fudan University, Shanghai, China.
Pediatr Rheumatol Online J. 2022 Jan 3;20(1):1. doi: 10.1186/s12969-021-00657-4.
The aim of this study was to evaluate demographic, clinical, laboratory, imaging, histopathology characteristics, and treatment responses of children with Chronic nonbacterial osteomyelitis (CNO).
Retrospective multi-center case series study of pediatric patients diagnosed with CNO treated at five tertiary centers in south China.
Totally there were 18 patients diagnosed as CNO between 2014 and 2020. The median age of onset was 9.2 years (range 3.7-13.1) and 55.6% were female. Median delay in diagnosis was 10.9 months (range 1.0-72.0). The most frequent presenting symptoms were bone pain (100%) and fever (44.4%). Most patients had more than one lesion (median of 5, range 1-7). Most frequently affected bones were tibiofibula (88.9%) and femur (77.8%). The MRI characteristics mainly presented as bone edema and hyperintensity in bone marrow. Bone biopsy was conducted in 11 patients (61.1%) with inflammatory cells infiltration manifested as chronic osteomyelitis, and none showed bacterial infection or tumor. In treatment, non-steroid anti-inflamatory drugs (NSAIDs) is used as the first-line drug followed by steriods, methotexate (MTX), salazosulfadimidine (SASP), Bisphosphonates and TNF-α inhibitor. Two refractory cases received combination therapy with Bisphosphonates and TNF-α inhibitor, and achieved good therapeutic effect.
The present study described a multicenter series of CNO from south China and highlighted the clinical features, laboratory tests, imaging characteristics and treatment outcomes. Increasing awareness of this disease is important to decrease time to diagnosis, improve access to treatment, and reduce complications.
本研究旨在评估儿童慢性非细菌性骨髓炎（CNO）的人口学、临床、实验室、影像学、组织病理学特征及治疗反应。
回顾性分析 2014 年至 2020 年间在华南五家三级中心诊断为 CNO 的儿科患者的多中心病例系列研究。
共诊断出 18 例 CNO 患儿，发病年龄中位数为 9.2 岁（范围 3.7-13.1），女性占 55.6%。中位诊断延迟时间为 10.9 个月（范围 1.0-72.0）。最常见的首发症状为骨痛（100%）和发热（44.4%）。大多数患者有一个以上的病变（中位数 5 个，范围 1-7 个）。最常受累的骨骼是胫腓骨（88.9%）和股骨（77.8%）。MRI 特征主要表现为骨髓水肿和信号增高。11 例（61.1%）患者进行了骨活检，表现为慢性骨髓炎的炎症细胞浸润，均无细菌感染或肿瘤。在治疗中，非甾体抗炎药（NSAIDs）作为一线药物，其次是类固醇、甲氨蝶呤（MTX）、柳氮磺胺吡啶（SASP）、双膦酸盐和 TNF-α抑制剂。2 例难治性病例接受了双膦酸盐和 TNF-α抑制剂联合治疗，取得了良好的治疗效果。
本研究描述了来自华南地区的一组多中心 CNO 病例，强调了该病的临床特征、实验室检查、影像学特征和治疗结果。提高对该病的认识对于缩短诊断时间、提高治疗机会和减少并发症非常重要。
