Ariza Jiménez Ana Belén, Núñez Cuadros Esmeralda, Galindo Zavala Rocío, Núñez Caro Leticia, Díaz-Cordobés Rego Gisela, Urda Cardona Antonio
UGC Pediatría, Hospital Materno-Infantil de Málaga, Málaga, España.
Unidad de Reumatología Pediátrica, Hospital Materno-Infantil de Málaga, Málaga, España.
Reumatol Clin (Engl Ed). 2018 Nov-Dec;14(6):334-338. doi: 10.1016/j.reuma.2017.04.002. Epub 2017 May 19.
Chronic recurrent multifocal osteomyelitis is a rare aseptic bone inflammation that affects pediatric patients. Its management and treatment have not yet been standardized.
Retrospective, descriptive study of patients under 14 years of age diagnosed with chronic nonbacterial osteomyelitis (CNBO) in a tertiary hospital. We included patients diagnosed over the last 6 years (2010-2015) who met the Jansson criteria. The clinical and radiological characteristics of CNBO were analyzed, as was the outcome after different therapeutic options.
We report 12 patients, with a mean age of 11 years (±1.6 standard deviation [SD]) and female predominance (10:2). The mean number of foci was 3.5 (±2.2 SD). The most common locations were ankle (58%), clavicle (50%), sternum (33%) and hip (25%). The mean disease duration was 10.5 months (±10.3 SD), and the median time to diagnosis was 2.38 months (range 0.17-16). Bone scintigraphy detected asymptomatic foci in 33% and we detected lytic lesions in 50% through magnetic resonance imaging. Biopsy was performed in 60%; 2/12 (16%) were associated with inflammatory disease and 1/12 (8.3%) later developed lymphoma. In all, 58% received antibiotic therapy with little response, 100% anti-inflammatory agents, 50% systemic corticosteroids, 41.6% methotrexate/pamidronate and 16% anti-tumor necrosis factor (TNF) α. The mean duration of treatment was 14.8 months (±12.4 SD) and 66% had recurrences. Currently, 83% are in clinical remission without treatment.
When CNBO is refractory to treatment with anti-inflammatory drugs, intravenous pamidronate can be an alternative. Anti-TNF drugs can be considered in patients who fail with pamidronate, as can agents associated with other autoimmune conditions.
慢性复发性多灶性骨髓炎是一种影响儿童患者的罕见无菌性骨炎症。其管理和治疗尚未标准化。
对一家三级医院中诊断为慢性非细菌性骨髓炎(CNBO)的14岁以下患者进行回顾性描述性研究。我们纳入了过去6年(2010 - 2015年)诊断且符合扬松标准的患者。分析了CNBO的临床和放射学特征以及不同治疗方案后的结局。
我们报告了12例患者,平均年龄11岁(±1.6标准差[SD]),女性占优势(10:2)。病灶平均数量为3.5个(±2.2 SD)。最常见的部位是踝关节(58%)、锁骨(50%)、胸骨(33%)和髋关节(25%)。平均病程为10.5个月(±10.3 SD),诊断的中位时间为2.38个月(范围0.17 - 16个月)。骨闪烁显像在33%的患者中检测到无症状病灶,通过磁共振成像在50%的患者中检测到溶骨性病变。60%的患者进行了活检;2/12(16%)与炎症性疾病相关,1/12(8.3%)后来发展为淋巴瘤。总体而言,58%的患者接受抗生素治疗但反应不佳,100%接受抗炎药治疗,50%接受全身糖皮质激素治疗,41.6%接受甲氨蝶呤/帕米膦酸治疗,16%接受抗肿瘤坏死因子(TNF)α治疗。平均治疗持续时间为14.8个月(±12.4 SD),66%的患者复发。目前,83%的患者未经治疗处于临床缓解状态。
当CNBO对抗炎药物治疗无效时,静脉注射帕米膦酸可能是一种选择。对于帕米膦酸治疗失败的患者,可以考虑使用抗TNF药物,与其他自身免疫性疾病相关的药物也可考虑。