以帕米膦酸盐疗法为重点的慢性非细菌性骨髓炎(CNO)和慢性复发性多灶性骨髓炎(CRMO)。

Pub Date : 2024-09-01 Epub Date: 2024-04-27 DOI:10.5507/bp.2024.007
Katerina Bouchalova, Zuzana Pytelova
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引用次数: 0

摘要

慢性复发性多灶性骨髓炎(CRMO)又称慢性非细菌性骨髓炎(CNO)或非细菌性骨髓炎(NBO),是一种病因不明的罕见自身炎症性骨病。然而,随着对该疾病的进一步了解,得到正确诊断的患者人数将会增加。在这方面,全身磁共振成像(WB MRI)比临床检查更能预测活动性病变。重要的是,RINBO 指数(非细菌性鼻炎的放射学指数)可根据 WB MRI 对受累情况进行量化。此外,慢性非细菌性骨髓炎核磁共振成像评分(CROMRIS)已被开发为评估 WB 核磁共振成像的在线工具。治疗包括非甾体抗炎药(NSAIDs)、双膦酸盐(帕米膦酸盐、唑来膦酸盐等)和其他药物,包括生物制剂。帕米膦酸钠是一种合适而安全的疗法。第一项帕米膦酸钠与安慰剂的前瞻性随机对照试验(RCT)是在成人中进行的。目前还没有针对儿童的研究。除了随机对照试验外,今后还有许多问题需要探讨,如治疗效果的预测因素、最佳治疗时间、治疗中断的预测因素以及最佳治疗方案的评估。最近,CNO 临床疾病活动性评分(CDAS)已经建立并通过验证,但分类标准仍在制定中。由于这种罕见疾病的合作至关重要,因此建立了一个前瞻性的慢性非细菌性骨髓炎国际登记处(CHOIR),以生成未来的比较效果研究数据。
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Chronic non-bacterial osteomyelitis (CNO) and chronic recurrent multifocal osteomyelitis (CRMO) with a focus on pamidronate therapy.

Chronic recurrent multifocal osteomyelitis (CRMO), also called chronic nonbacterial osteomyelitis (CNO) or nonbacterial osteomyelitis (NBO), is a rare autoinflammatory bone disease of unknown etiology. However, the number of patients properly diagnosed would increase with better knowledge of the disease. In this regard, whole-body magnetic resonance imaging (WB MRI) has been found to be a better predictor of active lesions than clinical examination. Importantly, the RINBO index (radiologic index for NBO) quantifies the involvement based on the WB MRI. Further, a chronic nonbacterial osteomyelitis MRI scoring (CROMRIS) has been developed as an online tool for assessing WB MRI. The therapy consists of non-steroidal anti-inflammatory drugs (NSAIDs), bisphosphonates (pamidronate, zoledronate, etc.) and other drugs, including biologics. Pamidronate is an appropriate and safe therapy. The first pilot prospective randomised controlled trial (RCT) on pamidronate vs. placebo was carried out in adults. No RCT has been done in children yet. Besides RCTs, there are a number of issues to be explored in future, i.e. predictors of therapy effect, optimal therapy duration, predictors of therapy discontinuation and evaluation of optimal therapy protocol. Recently, the CNO clinical disease activity score (CDAS) was constructed and validated but the classification criteria are still being developed. As collaboration on this rare disease is essential, a prospective Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) was established to generate future comparative effectiveness research data.

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