背景:慢性非细菌性骨髓炎(CNO)是一种罕见的,以及儿童和成人发生的有影响的自身炎症性骨病。CNO的临床护理具有挑战性,由于该疾病缺乏经过验证的分类标准和循证治疗。这项研究旨在绘制成人CNO的当前诊断和治疗实践图,作为迈向标准化疾病定义和未来共识治疗计划的第一步。
方法:一项主要调查在全球风湿病/骨骼网络中进行,并从文献中确定了57位专家(2022年5月),涵盖术语,诊断工具(临床,放射学,生化)和处理步骤。二次调查(于2022年8月发送给主要调查响应者)进一步查询了关键诊断功能,治疗动机,疾病活动和治疗反应监测。
结果:36名和23名医生分别完成了初级和次级调查。诊断主要基于医生的个人评估,其中慢性复发性骨痛与放射学证实的骨炎/骨髓炎的组合,硬化症,据报道,骨闪烁显像上的骨肥大和同位素吸收增加表明了CNO。医生似乎更有可能将这种情况称为滑膜炎,痤疮,脓疱病,骨增生,骨炎(SAPHO)综合征存在关节和皮肤病理。MRI是最常见的,和首选诊断测试为47%。X射线是第二常用的,尽管被认为是所有可用工具中信息最少的。报告的典型影像学特征是骨肥大,骨炎,骨硬化,骨髓水肿,而退化,软组织钙化,强直并不被认为是特征性的。炎症标志物和骨标志物通常被认为对诊断和监测目的没有帮助,并且医师很少进行骨活检。管理策略分歧,包括治疗适应症,反应监测和缓解声明。第1步治疗包括非甾体抗炎药/COX-2抑制剂(83%)。常见的第2-3步治疗是帕米膦酸盐,甲氨蝶呤,和TNF-α抑制(抗TNFα),后两者认为在SAPHO综合征中联合靶向骨外炎症特别方便.总体帕米膦酸盐和抗TNFα被认为是最有效的治疗。
结论:根据我们的调查数据,成人CNO是一种广泛且特征不充分的疾病谱,包括骨外特征。MRI是最受欢迎的影像诊断,和管理策略差异很大。总的来说,帕米膦酸盐和抗TNFα被认为是最成功的。结果列出了成人CNO的当前做法,这可能是未来共识临床指南的支柱。
Chronic nonbacterial osteomyelitis (CNO) is a rare, and impactful auto-inflammatory bone disease occurring in children and adults. Clinical care for CNO is challenging, as the condition lacks validated classification criteria and evidence-based therapies. This study aimed to map the current diagnostic and therapeutic practices for CNO in adults, as a first step towards a standardized disease definition and future consensus treatment plans.
A primary survey was spread among global rheumatological/bone networks and 57 experts as identified from literature (May 2022), covering terminology, diagnostic tools (clinical, radiological, biochemical) and treatment steps. A secondary survey (sent to primary survey responders in August 2022) further queried key diagnostic features, treatment motivations, disease activity and treatment response monitoring.
36 and 23 physicians completed the primary and secondary survey respectively. Diagnosis was mainly based on individual physician assessment, in which the combination of chronic relapsing-remitting bone pain with radiologically-proven osteitis/osteomyelitis, sclerosis, hyperostosis and increased isotope uptake on bone scintigraphy were reported indicative of CNO. Physicians appeared more likely to refer to the condition as synovitis, acne, pustulosis, hyperostosis, osteitis (
SAPHO) syndrome in the presence of joint and skin pathology. MRI was most frequently performed, and the preferred diagnostic test for 47%. X-rays were second-most frequently used, although considered least informative of all available tools. Typical imaging features reported were hyperostosis, osteitis, osteosclerosis, bone marrow edema, while degeneration, soft tissue calcification, and ankylosis were not regarded characteristic. Inflammation markers and bone markers were generally regarded unhelpful for diagnostic and monitoring purposes and physicians infrequently performed bone biopsies. Management strategies diverged, including indications for treatment, response monitoring and declaration of remission. Step-1 treatment consisted of non-steroidal anti-inflammatory drugs/COX-2 inhibitors (83%). Common step 2-3 treatments were pamidronate, methotrexate, and TNF-a-inhibition (anti-TNFα), the latter two regarded especially convenient to co-target extra-skeletal inflammation in
SAPHO syndrome. Overall pamidronate and anti-TNFα and were considered the most effective treatments.
Following from our survey data, adult CNO is a broad and insufficiently characterized disease spectrum, including extra-osseous features. MRI is the favoured imaging diagnostic, and management strategies vary significantly. Overall, pamidronate and anti-TNFα are regarded most successful. The results lay out current practices for adult CNO, which may serve as backbone for a future consensus clinical guideline.