Objectives Primary chronic Non-Bacterial Osteomyelitis of the jaw is a rare auto-inflammatory disease of unknown aetiology that bears pathophysiological resemblance to both the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome in adults and chronic recurrent multifocal osteomyelitis (CRMO) in children. Both SAPHO and CRMO respond to TNF-alpha blockade. Previously reported treatment regimens in CNOM including non-steroidal anti-inflammatory drugs, corticosteroids, antibiotics, anti-resorptive therapy, and surgery all bear disappointing results. TNF- α blockade is suggested as a treatment option by some experts but this is not backed by any clinical data. We sought to retrospectively and exhaustively report our experience of anti-TNF alpha therapy in refractory CNOM. Methods Fifteen patients with refractory CNOM and high disease burden were referred to our centre. TNF- α blockade was attempted in 10 cases, given its efficacy in neighbouring diseases, its good tolerance profile and failure of previous treatment strategies We herein retrospectively report detailed outcomes for all patients having received anti-TNF alpha therapy for this indication in our centre. Results TNF-α-targeting therapy resulted in a rapid and sustained remission in a majority of patients with CNOM, without serious adverse events. Treatment was tapered and stopped without relapse in some patients despite a refractory course of several years. Male sex seems to be associated with a poorer outcome. Conclusion Our results suggest that blocking TNF-α is efficient and safe in CNOM

中文翻译：

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TNF-α 阻断治疗原发性慢性非细菌性下颌骨骨髓炎


目的 原发性慢性颌骨非细菌性骨髓炎是一种病因不明的罕见自身炎症性疾病，其病理生理学与成人滑膜炎、痤疮、脓疱病、骨质增生和骨炎 (SAPHO) 综合征以及慢性复发性多灶性骨髓炎 (CRMO) 相似。 ）在儿童中。 SAPHO 和 CRMO 均对 TNF-α 阻断有反应。此前报道的CNOM治疗方案包括非甾体抗炎药、皮质类固醇、抗生素、抗骨吸收治疗和手术，但结果均令人失望。一些专家建议将 TNF-α 阻断作为一种治疗选择，但这没有得到任何临床数据的支持。我们试图回顾性地、详尽地报告我们在难治性 CNOM 中抗 TNF α 治疗的经验。方法 15 例难治性 CNOM 且疾病负担高的患者被转诊至我中心。考虑到 TNF-α 阻断对邻近疾病的疗效、良好的耐受性以及之前治疗策略的失败，我们在 10 例病例中尝试了 TNF-α 阻断。我们在此回顾性地报告了在我们中心针对该适应症接受抗 TNF-α 治疗的所有患者的详细结果。结果 TNF-α 靶向治疗使大多数 CNOM 患者快速持续缓解，且没有严重不良事件。尽管有数年的难治性病程，但一些患者的治疗逐渐减少并停止，没有复发。男性似乎与较差的结果有关。结论 我们的结果表明，阻断 TNF-α 在 CNOM 中是有效且安全的