S. Aden, BS, C. Yang, BS, T. Bui, BS, University of Washington.
S. Wong, MD, Y. Zhao, MD, PhD, Seattle Children's Hospital, and Center of Clinical and Translational Research, Seattle Children's Research Institute.
J Rheumatol. 2022 Aug;49(8):929-934. doi: 10.3899/jrheum.210991. Epub 2022 Mar 15.
Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease that is gaining recognition from clinicians and researchers. We aim to publish data from our cohort of patients with CNO living in the northwestern United States to increase the awareness of specific demographics, characteristics, and presentation of this rare disease.
A retrospective chart review was performed of our electronic medical records. Patients with complete chart records who met criteria for a diagnosis of CNO from 2005 to 2019 were included. Extracted data including patient demographics, bone biopsy results, and lesion locations on advanced imaging were analyzed. King County census data were used to calculate the annual new case rate within our center.
A total of 215 CNO cases were diagnosed at our large tertiary pediatric hospital. The majority of cases were of White race residing in Washington's most populous county, King County. Most cases were diagnosed in 2016 to 2019, showing a significant increase in the annual case rate from 8 to 23 per million children in King County, though there did not appear to be a seasonal predilection. Biopsy rate decreased from 75% to 52%. One hundred fifty-two (71%) children had family history of autoimmunity. With increasing use of whole-body magnetic resonance imaging (WB-MRI), results showed 68% had multiple lesions.
CNO has been diagnosed at an increased rate in recent years. WB-MRI may assist in identifying other lesions that may be asymptomatic on presentation. Bone biopsy is still required in some children at the time of diagnosis.
慢性非细菌性骨髓炎(CNO)是一种罕见的自身炎症性骨病,正在引起临床医生和研究人员的关注。我们旨在发表我们在美国西北部的 CNO 患者队列的数据,以提高对这种罕见疾病的特定人群、特征和表现的认识。
对我们的电子病历进行了回顾性图表审查。符合 2005 年至 2019 年 CNO 诊断标准且病历完整的患者被纳入研究。提取的数据包括患者人口统计学、骨活检结果以及高级影像学上的病变部位。使用金县的人口普查数据计算我们中心的年度新发病例率。
在我们的大型三级儿科医院共诊断出 215 例 CNO 病例。大多数病例为白人,居住在华盛顿人口最多的金县。大多数病例是在 2016 年至 2019 年诊断的,表明金县的年度病例率从每百万儿童 8 例显著增加到 23 例,尽管似乎没有季节性倾向。活检率从 75%下降到 52%。152 名(71%)儿童有自身免疫性疾病家族史。随着全身磁共振成像(WB-MRI)的广泛应用,结果显示 68%的儿童有多个病变。
近年来,CNO 的诊断率有所增加。WB-MRI 可能有助于识别在就诊时可能无症状的其他病变。在某些儿童诊断时仍需要进行骨活检。