Abstract：
Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease that primarily affects children and adolescents. CNO is associated with pain, bone swelling, deformity, and fractures. Its pathophysiology is characterized by increased inflammasome assembly and imbalanced expression of cytokines. Treatment is currently based on personal experience, case series and resulting expert recommendations. Randomized controlled trials (RCTs) have not been initiated because of the rarity of CNO, expired patent protection of some medications, and the absence of agreed outcome measures. An international group of fourteen CNO experts and two patient/parent representatives was assembled to generate consensus to inform and conduct future RCTs. The exercise delivered consensus inclusion and exclusion criteria, patent protected (excludes TNF inhibitors) treatments of immediate interest (biological DMARDs targeting IL-1 and IL-17), primary (improvement of pain; physician global assessment) and secondary endpoints (improved MRI; improved PedCNO score which includes physician and patient global scores) for future RCTs in CNO.
摘要：
慢性非细菌性骨髓炎（CNO）是一种主要影响儿童和青少年的自身炎症性骨病。CNO与疼痛有关,骨肿胀,畸形,和骨折。其病理生理学特征在于增加的炎症小体组装和细胞因子的不平衡表达。目前的治疗是基于个人经验，案例系列和由此产生的专家建议。随机对照试验（RCT）尚未启动，因为CNO的稀有性，某些药物的专利保护过期，以及没有商定的结果措施。一个由14名CNO专家和两名患者/家长代表组成的国际小组聚集在一起，以达成共识，为未来的RCT提供信息和进行。这项工作达成了共识的纳入和排除标准，专利保护（不包括TNF抑制剂）直接感兴趣的治疗（靶向IL-1和IL-17的生物DMARD），主要终点(疼痛改善;医师整体评估)和次要终点(改善MRI;改善PedCNO评分,包括医师和患者整体评分)用于未来CNO的RCTs.
