From the Department of Rheumatology, the Department of Radiology, and the Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital; the Department of Clinical Medicine, Aarhus University, Aarhus, Denmark.
C.M. Andreasen, MD, PhD, Department of Rheumatology, and the Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital; A.G. Jurik, MD, DMSc, Department of Radiology, Aarhus University Hospital, and the Department of Clinical Medicine, Aarhus University; M.B. Glerup, MD, Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital; C. Høst, MD, PhD, Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital; B.T. Mahler, MD, PhD, Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital; E.M. Hauge, MD, PhD, Department of Rheumatology, Aarhus University Hospital, and the Department of Clinical Medicine, Aarhus University; T. Herlin, MD, DMSc, Department of Clinical Medicine, Aarhus University, and the Department of Pediatrics and Adolescent Medicine, Aarhus University Hospital.
J Rheumatol. 2019 Nov;46(11):1515-1523. doi: 10.3899/jrheum.181254. Epub 2019 Apr 15.
Chronic nonbacterial osteomyelitis (CNO) is a sterile inflammatory bone disorder with an unpredictable disease course. The objective was to assess clinical and radiological disease activity in children with CNO including response to early-onset pamidronate treatment.
A single-center retrospective study was conducted of children fulfilling the Bristol Criteria for CNO. At the time of diagnosis, whole-body magnetic resonance imaging (WB-MRI) or local MRI was performed to assess radiological disease activity. Children with multifocal or spinal bone inflammation and clinical disease activity not responding to nonsteroidal antiinflammatory drugs were categorized as having extended CNO. Clinical disease activity was assessed annually.
Fifty-one children were included. Median followup time was 4 years (interquartile range 3-7). Children categorized with extended CNO (n = 32) were treated in an early-onset 2-year pamidronate regimen. In extended CNO, WB-MRI was performed at time of diagnosis, and at years 1 and 2 in 88%, 84%, and 91% of cases, respectively. During the first year, the total number of radiologically active lesions and number of spinal lesions per patient declined (p = 0.01). Clinically inactive disease was recorded in 12/32 children (38%). However, 8/12 children (67%) experienced clinical relapse. In limited CNO (n = 19), 10/19 children (53%) presented with clinically inactive disease after 1 year and did not experience clinical relapse.
Pamidronate might contribute to improvement in clinical and radiological disease activity in extended CNO, especially after 1 year of treatment. However, children with continuously active disease after 2 years of pamidronate treatment were seen.
慢性非细菌性骨髓炎（CNO）是一种无菌性炎症性骨病，其病程不可预测。本研究旨在评估儿童 CNO 的临床和放射学疾病活动，包括对早期帕米膦酸盐治疗的反应。
对符合布里斯托 CNO 标准的儿童进行单中心回顾性研究。在诊断时，行全身磁共振成像（WB-MRI）或局部 MRI 以评估放射学疾病活动。将有多发性或脊柱骨炎症且对非甾体抗炎药无临床疾病反应的儿童归类为扩展型 CNO。每年评估临床疾病活动。
共纳入 51 例儿童。中位随访时间为 4 年（四分位距 3-7 年）。32 例扩展型 CNO 患儿接受了早期 2 年帕米膦酸盐治疗方案。在扩展型 CNO 中，88%、84%和 91%的病例分别在诊断时、第 1 年和第 2 年进行了 WB-MRI。在第 1 年，患者的放射学活跃病变总数和脊柱病变数均减少（p = 0.01）。12/32 例（38%）患儿记录为临床无活动疾病。然而，8/12 例（67%）患儿出现临床复发。在局限性 CNO（n = 19）中，10/19 例（53%）患儿在第 1 年出现临床无活动疾病，且无临床复发。
帕米膦酸盐可能有助于改善扩展型 CNO 的临床和放射学疾病活动，尤其是在治疗 1 年后。然而，在接受 2 年帕米膦酸盐治疗后，仍有患儿出现持续活跃的疾病。
