Saint-Petersburg State Pediatric Medical University, Lytovskaya 2, Saint-Petersburg, Russia, 194100.
Almazov National Medical Research Center, Saint-Petersburg, Russia.
Rheumatol Int. 2020 Jan;40(1):97-105. doi: 10.1007/s00296-019-04479-2. Epub 2019 Nov 21.
Chronic non-bacterial osteomyelitis (CNO) is a group of immune-mediated diseases which appears in bone inflammation, destruction and some orthopaedic consequences, especially in the cases of spinal involvement. This study is to compare characteristics and treatment outcomes of CNO patients with spinal involvement. The retrospective cohort study included data from 91 pediatric patients with CNO. The diagnosis is based on Jannson's criteria with morphological confirmation (nonspecific chronic inflammation). Spine involvement detected by X-ray, computed tomography, magnetic resonance imaging, and bone scan in 29 (31.9%) patients. No differences in the family history, concomitant immune-mediated diseases between spinal (SpCNO) and peripheral (pCNO) forms of CNO have been revealed. Only 5 (10.2%) SpCNO patients (10.2%) had monofocal monovertebral involvement. The main risk factors of spinal involvement were female sex: RR = 2.0 (1.1; 3.9), sensitivity (Se) = 0.66, specificity (Sp) = 0.6; multifocal involvement: RR = 2.1 (0.9; 5.0), Se = 0.83, Sp = 0.37; no foot bones involvement: RR = 3.1 (1.3; 7.5), Se = 0.83, Sp = 0.5; sternum involvement RR = 2.3 (1.3; 4.1), Se = 0.24, Sp = 0.94. In the linear regression analysis only female sex (p = 0.005), multifocal involvement (p = 0.000001) and absence of foot bones involvement (p = 0.000001) were independent risk factors of spinal involvement (p = 0.000001). The response rate on bisphosphonates and tumor necrosis factor-a inhibitors was 90.9% and 66.7%, consequently. Only 4/29 (13.8%) SpCNO patients underwent surgery due to severe spinal instability or deformities. The spinal involvement is frequent in CNO and could be crucial for choosing a treatment strategy. Bisphosphonates and TNFa-inhibitors could be effective treatment options for severe SpCNO.
慢性非细菌性骨髓炎(CNO)是一组免疫介导的疾病,表现为骨炎症、破坏和一些骨科后果,特别是在脊柱受累的情况下。本研究旨在比较伴有脊柱受累的 CNO 患者的特征和治疗结果。这项回顾性队列研究纳入了 91 例 CNO 儿科患者的数据。诊断基于 Jannson 标准,并通过形态学确认(非特异性慢性炎症)。29 例(31.9%)患者通过 X 射线、计算机断层扫描、磁共振成像和骨扫描检测到脊柱受累。脊柱(SpCNO)和外周(pCNO)形式的 CNO 之间在家族史和合并免疫介导疾病方面无差异。仅有 5 例(10.2%)SpCNO 患者(10.2%)存在单灶单椎体受累。脊柱受累的主要危险因素是女性:RR=2.0(1.1;3.9),敏感性(Se)=0.66,特异性(Sp)=0.6;多灶性受累:RR=2.1(0.9;5.0),Se=0.83,Sp=0.37;无足部骨骼受累:RR=3.1(1.3;7.5),Se=0.83,Sp=0.5;胸骨受累 RR=2.3(1.3;4.1),Se=0.24,Sp=0.94。在线性回归分析中,只有女性(p=0.005)、多灶性受累(p=0.000001)和无足部骨骼受累(p=0.000001)是脊柱受累的独立危险因素(p=0.000001)。双膦酸盐和肿瘤坏死因子-α抑制剂的反应率分别为 90.9%和 66.7%。仅 4/29(13.8%)例 SpCNO 患者因严重脊柱不稳定或畸形而行手术治疗。脊柱受累在 CNO 中很常见,对于选择治疗策略至关重要。双膦酸盐和 TNFa 抑制剂可能是严重 SpCNO 的有效治疗选择。