成年慢性非细菌性骨髓炎 (CNO) 的诊断和治疗实践
A T Leerling1,2, G Clunie3, E Koutrouba1,2
1Department of Internal Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands.
Orphanet journal of rare diseases
|July 21, 2023
概括
慢性非细菌性骨髓炎 (CNO) 是一种罕见的骨疾病,缺乏明确的诊断标准. 目前的成年CNO实践有所不同,MRI是诊断的优势,帕米德罗纳特/抗TNFα显示有效性.
科学领域:
- 类风湿病学 类风湿病学
- 整形外科 整形外科 整形外科
- 自发炎性疾病 自发炎性疾病
背景情况:
- 慢性非细菌性骨髓炎 (CNO) 是一种罕见的,具有影响力的儿童和成人自身炎症性骨疾病.
- 由于缺乏有效的分类标准和基于证据的治疗方法,临床护理具有挑战性.
- 目前的研究绘制了成年CNO的诊断和治疗实践图,以提供标准化的定义和治疗方法.
研究的目的:
- 绘制成年人慢性非细菌性骨髓炎 (CNO) 的当前诊断和治疗实践.
- 建立一个标准化疾病定义的基础.
- 为指导未来共识治疗计划的制定提供指导.
主要方法:
- 全球风湿学/骨专家 (n=57) 的调查分为两阶段 (2022年5月和8月) 进行.
- 该调查涵盖了CNO的术语,诊断工具 (临床,放射学,生物化学) 和治疗策略.
- 分析了36名 (初级) 和23名 (二级) 医生的数据.
主要成果:
- 诊断依赖于医生的评估,将慢性骨痛与像骨质炎和超静止症这样的成像发现相结合.
- 磁力共振成像是首选的诊断测试 (47%),而X射线信息较少.
- 管理策略有所不同;NSAIDs/COX-2抑制剂是第一线的,帕米德罗纳酸和抗TNFα被认为是2-3阶段治疗中最有效的.
结论:
- 成人CNO代表了一种广泛的,不够表征的疾病谱,具有骨外特征.
- 核磁共振是最受青的诊断成像方式,但医生之间管理策略有很大的不同.
- 帕米德罗纳酸和抗TNFα被认为是最成功的治疗方法,为未来的临床指南提供了基础.
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