儿童慢性无菌性骨髓炎8例并文献复习

Report of 8 cases with pediatric chronic non-bacterial osteomyelitis and literature review

目的总结儿童慢性无菌性骨髓炎(CNO)的临床特点。方法收集2014年3月至2020年12月首都儿科研究所附属儿童医院风湿免疫科诊治的8例CNO患儿的临床资料并进行回顾性分析,总结8例患儿的临床特点,并与国外报道的儿童CNO患者进行比较和文献复习。结果8例患儿,男3例,女5例;发病年龄(7.2±3.2)岁,平均确诊时间25.9个月;常见临床症状为骨痛(7例,87.5%),关节炎(4例,50.0%),发热(3例,37.5%);X线片与CT主要表现为骨质破坏、进行性骨质硬化;磁共振成像(MRI)主要表现为骨髓水肿、骨膜炎、软组织肿胀,增强扫描可见强化;患儿受累骨骼均>1处,7例(87.5%)患儿为双侧病灶;常见部位为胫骨(22.0%),其次为股骨(17.1%)、下颌骨(9.8%);8例患儿均行病变部位骨活检,其中4例表现为骨坏死,4例骨纤维化改变,2例骨髓炎改变;骨组织病原学检查均阴性;8例患儿接受非甾类抗炎药单药或联合糖皮质激素、慢作用抗风湿药、双膦酸盐及肿瘤坏死因子ɑ拮抗剂等药物治疗后随访3个月~2年,8例患儿病情均好转,监测炎症指标正常,均无致残致畸和多器官损伤。结论儿童CNO多于学龄期起病,病程长;以多部位骨痛、关节炎为主要表现;影像学可见多处骨骼受累,以下肢骨最常见;非甾体类抗炎药、糖皮质激素、慢作用抗风湿药、双膦酸盐及肿瘤坏死因子α拮抗剂对CNO有效。

Objective To summarize the clinical features of chronic non-bacterial osteomyelitis(CNO)in children.Methods Clinical data of 8 CNO patients admitted to the Department of Rheumatology and Immunology,Children′s Hospital Affiliated to Capital Institute of Pediatrics from March 2014 to December 2020 were retrospectively analyzed.The clinical characteristics of 8 children with CNO were summarized and compared with those reported abroad.Results A total of 8 CNO patients were recruited,involving 3 males and 5 females with the mean age of onset(7.2±3.2)years,and the average diagnosis time 25.9 months,respectively.The common clinical symptoms included bone pain(7 cases,87.5%),arthritis(4 cases,50.0%),and fever(3 cases,37.5%).The main manifestations on X-ray and CT scans were bone destruction and progressive osteosclerosis.Magnetic resonance imaging(MRI)showed bone marrow edema,periostitis,soft tissue swelling,and enhancement.All of them had more than one site of bone involvement.Seven patients(87.5%)had bilateral bone involvement,with the most common site of tibia(22.0%),followed by femur(17.1%)and mandible(9.8%).Bone biopsy was performed in 8 patients,and 4 cases showed osteonecrosis,4 cases showed bone fibrosis and 2 cases showed osteomyelitis.The etiological examination of the bone was negative.Eight children received non-steroid anti-inflammatory drugs alone or in combination with glucocorticoids,disease-modifying antirheumatic drugs(DMARDs),bisphosphonates or tumor necrosis factor-α(TNF-α)antagonists.After treatment,the patients were followed up for 3 months to 2 years.Eight children improved.Their inflammatory indexes were normal,and had no disability,teratology or multiple organ damage.Conclusions Pediatric CNO is more common in children of school age,with a long course of disease.The main manifestations are multi-site bone pain and arthritis.Imaging studies indicate multiple bone involvement,which is more common at lower extremities.Non-steroids anti-inflammatory drugs,glucocorticoids,DMARDs,bisphosphonates and TNF-αantagonists are effective to CNO.