当前和未来的实践进展:SAPHO 综合征和慢性非细菌性骨炎 (CNO)。

IF 2.1 Q3 RHEUMATOLOGY
Rheumatology Advances in Practice Pub Date : 2024-10-14 eCollection Date: 2024-01-01 DOI:10.1093/rap/rkae114
Victoria Furer, Mitsumasa Kishimoto, Tetsuya Tomita, Ori Elkayam, Philip S Helliwell
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引用次数: 0

摘要

滑膜炎、痤疮、脓疱病、骨质增生和骨炎(SAPHO)综合征是一种罕见的、诊断不足的疾病,临床表现广泛。无菌性骨炎(主要发生在前胸)和皮肤表现(掌跖脓疱病、寻常型银屑病和痤疮)是 SAPHO 的主要特征,与 SpA 有某些相似之处。SAPHO 与小儿慢性非细菌性骨炎(CNO)密切相关,后者是一种自身炎症性骨病。SAPHO 的病因被认为是遗传、免疫和感染等复杂因素相互作用的多因素结果。尽管人们对 SAPHO/CNO 的认识不断提高,但由于缺乏有效的分类和诊断标准,延误诊断的情况十分普遍。SAPHO 的治疗是一项挑战,包括抗炎药物、抗生素、双磷酸盐、合成的传统 DMARDs 以及标签外使用的抗细胞因子生物制剂和 Janus 激酶抑制剂。本综述总结了 SAPHO/CNO 目前的诊断和实际治疗方法,并重点介绍了正在进行的有关诊断标准、核心领域和治疗方法的定义和验证方面的研究工作。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Current and future advances in practice: SAPHO syndrome and chronic non-bacterial osteitis (CNO).

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, underdiagnosed disease with a wide clinical spectrum. Sterile bone inflammation, predominantly of the anterior chest, and skin manifestations (palmoplantar pustulosis, psoriasis vulgaris and acne) are the key features of SAPHO, which shares certain similarities with SpA. SAPHO is closely related to paediatric chronic non-bacterial osteitis (CNO), a spectrum of autoinflammatory bone diseases. The aetiology of SAPHO is considered multifactorial based on a complex interplay of genetic, immune and infectious factors. Despite the increasing awareness of SAPHO/CNO, diagnostic delay is common, as validated classification and diagnostic criteria are lacking. Treatment of SAPHO represents a challenge and includes anti-inflammatory drugs, antibiotics, bisphosphonates, synthetic conventional DMARDs and off-label use of anti-cytokine biologics and Janus kinase inhibitors. This review summarizes the current diagnostic and practical treatment approach to SAPHO/CNO and highlights the ongoing research endeavours concerning the definition and validation of diagnostic criteria, core domains and treatment.

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来源期刊
Rheumatology Advances in Practice
Rheumatology Advances in Practice Medicine-Rheumatology
CiteScore
3.60
自引率
3.20%
发文量
197
审稿时长
11 weeks
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