Department of Infectious Diseases and Pediatric Immunology, School of Medicine, Pontificia Universidad Católica de Chile, Diagonal Paraguay 362 Of. 807, Santiago, 8330077, Chile.
Department of Pediatric Immunology and Rheumatology, Hospital Hernán Henríquez Aravena, Temuco, Chile.
Rheumatol Int. 2020 Jan;40(1):115-120. doi: 10.1007/s00296-019-04400-x. Epub 2019 Aug 2.
Chronic nonbacterial osteomyelitis (CNO) is a primary autoinflammatory bone disease that presents more frequently in children and is characterized by inflammatory bone lesions in the absence of an infectious etiology. There is little information of this disease in Latin America. The objective of the study was to evaluate demographic, clinical, laboratory, imaging, histopathology characteristics, and treatment responses of pediatric CNO patients. The clinical records of 19 patients with CNO diagnosed between 2007 and 2019 at three tertiary centers in Santiago, Chile were reviewed. The median age of onset was 10 years and 47% were female. Median delay in diagnosis was 12 months. All patients had a pattern of recurrent multifocal disease. 37% of patients had positive antinuclear antibodies and 16% HLA-B27 positivity. 21% of patients presented arthritis or other rheumatologic comorbidity, although no association with psoriasis, inflammatory bowel disease (IBD) or palmoplantar pustulosis (PPP) was observed. Eighteen patients received treatment with nonsteroidal anti-inflammatory drugs with partial response. Twelve patients received methotrexate, and half of them received steroids at the same time reaching remission in 50%. Of the five patients who received bisphosphonates, 60% achieved remission. All four patients who received adalimumab had comorbid arthritis and 75% achieved remission. In a series of Chilean children with CNO, all patients presented with multifocal lesions. Comorbid autoimmune diseases including arthritis were frequent, but no association was observed with psoriasis, IBD, or PPP.
慢性非细菌性骨髓炎 (CNO) 是一种主要的自身炎症性骨病，更常发生于儿童，其特征是在无感染病因的情况下出现炎症性骨病变。拉丁美洲对此病的了解甚少。本研究的目的是评估智利圣地亚哥的三个三级中心的 19 例儿科 CNO 患者的人口统计学、临床、实验室、影像学、组织病理学特征和治疗反应。回顾了 2007 年至 2019 年间在这三个三级中心诊断为 CNO 的 19 例患者的临床记录。发病中位年龄为 10 岁，47%为女性。中位诊断延迟时间为 12 个月。所有患者均存在复发性多灶性疾病模式。37%的患者存在抗核抗体阳性，16%的患者 HLA-B27 阳性。21%的患者出现关节炎或其他风湿性合并症，但未观察到与银屑病、炎症性肠病（IBD）或掌跖脓疱病（PPP）有关。18 例患者接受非甾体抗炎药治疗，部分缓解。12 例患者接受甲氨蝶呤治疗，其中一半同时接受皮质类固醇治疗，50%达到缓解。接受双膦酸盐治疗的 5 例患者中，有 60%达到缓解。接受阿达木单抗治疗的 4 例患者均有合并关节炎，缓解率为 75%。在智利一组 CNO 患儿中，所有患者均存在多灶性病变。合并自身免疫性疾病，包括关节炎，较为常见，但未观察到与银屑病、IBD 或 PPP 相关。
