慢性无菌性骨髓炎18例临床特点

Clinical characteristics of 18 children with chronic nonbacterial osteomyelitis

目的总结儿童慢性无菌性骨髓炎(CNO)的临床特点并提高临床医生的认识。方法回顾性分析2015年1月至2021年12月复旦大学附属儿科医院风湿科确诊的18例CNO患儿的临床特点。结果18例患儿中男12例、女6例,发病年龄为9(5,11)岁,症状出现至确诊时间为2(1,6)个月,随访时间17(8,34)个月。14例患儿发热起病,14例骨痛和(或)关节痛;17例外周血白细胞正常,18例红细胞沉降率(ESR)均升高,14例C反应蛋白(CRP)升高。2例抗核抗体阳性,18例人类白细胞抗原-B27和类风湿因子均为阴性。影像学提示18例骨骼受累病灶均为对称性、多病灶性,受累骨为8(6,11)个,较常受累部位为18例胫骨和17例股骨。14例患儿进行骨活检,病理表现均为急或慢性骨髓炎症改变。磁共振成像(MRI)发现骨病变为18例,同位素全身骨显像发现骨病变为13例。18例患儿均给予非甾体类抗炎药治疗,10例联合糖皮质激素,9例联合使用传统缓解病情抗风湿病药物,8例联合使用阿伦磷酸盐,6例联合使用肿瘤坏死因子拮抗剂。随访6个月,13例患儿达到儿童CNO疾病活动度评分70%及以上改善。结论CNO临床表现缺乏特异性,首发症状表现为发热,伴或不伴有骨痛和(或)关节痛,伴外周血白细胞正常,CRP和(或)ESR升高,全身骨扫描联合MRI可以早期发现亚临床部位的骨髓炎,提高CNO的诊断率。

Objective To investigate the clinical features of children with chronic nonbacterial osteomyelitis(CNO),and raise awareness among clinicians.Methods In this retrospective study,18 patients with CNO who were diagnosed in Children′s Hospital of Fudan University from January 2015 to December 2021 were included.Results Eighteen children with CNO(12 males,6 females)were identified.Their age at onset was 9(5,11)years,the delay in diagnosis was 2(1,6)months,and follow-up-was 17(8,34)months.The most common symptoms were fever in 14 children,as well as bone pain and(or)arthralgia in 14 children.In terms of laboratory results,normal white blood cell counts were observed at onset in 17 patients;increased erythrocyte sedimentation rate(ESR)in all patients;increased C reactive protein(CRP)over the normal value in 14 patients.Of the 18 patients,2 had positive antinuclear antibodies,while none had positive human leukocyte antigen-B27 or rheumatoid factor.Imaging examination revealed that all the patients had symmetrical and multifocal skeletal lesions.The number of structural lesions detected by imaging investigation was 8(6,11).The most frequently affected bones were tibia in 18 patients and femur in 17 patients.Bone biopsy was conducted in 14 patients and acute or chronic osteomyelitis manifested with inflammatory cells infiltration were detected.Magnetic resonance imaging(MRI)found bone lesions in all the patients and bone scintigraphy were positive in 13 patients.All the patients were treated with nonsteroidal anti-inflammatory drugs,among whom 10 cases also treated with oral glucocorticoids,9 cases with traditional disease modifying anti-rheumatic drugs,8 cases with bisphosphonates and 6 cases with tumor necrosis factor inhibitors.The pediatric chronic nonbacterial osteomyelitis disease activity score,increased by 70%or more in 13 patients within the initial 6-month follow-up.Conclusions The clinical manifestations of CNO are lack of specificity.The first symptom of CNO is fever,with or without bone pain and(or)arthralgia,with normal peripheral blood leukocytes,elevated CRP and(or)ESR.Whole body bone scanning combined with MRI can early detect osteomyelitis at subclinical sites,and improve the diagnostic rate of CNO.