Objectives Chronic nonbacterial osteomyelitis (CNO) is a rare inflammatory bone disease. The distinct CNO subtype that affects the anterior chest wall is descriptively named sternocostoclavicular hyperostosis (SCCH) and mainly occurs in adults. Literature on CNO/SCCH is scattered, and lacks diagnostic and therapeutic consensus. Methods Systematic review and meta-analysis aiming to characterize 1) clinical presentation and i2) therapeutic modalities applied in adult CNO/SCCH patients. Untransformed numerical data and double-arcsine transformed proportional data were pooled in a random effects model in R-4.0.5; proportions were reported with 95% confidence intervals(95%CI). Results 40 studies were included, containing data on 2030 and 642 patients for aim 1 and 2 respectively. A female predisposition (67%, 95%CI60-73) and major diagnostic delay (5 years 95%CI3-7) were noted. Clinical presentation included chest pain (89%, 95%CI79-96), and swelling (79%, 95%CI62-91). Patients suffered from pustulosis palmoplantaris (53%, 95%CI37-68), arthritis (24%, 95%CI 11–39) and acne (8%, 95%CI4-13). Inflammatory markers were inconsistently elevated. Autoantibody and HLA-B27 prevalence was normal, and histopathology unspecific. Increased isotope uptake (99%, 95%CI96-100) was a consistent imaging finding. Amongst manifold treatments, pamidronate and biologicals yielded good response in 83%, 95%CI60-98 and 56%, 95%CI26-85 respectively. Conclusion CNO/SCCH literature proves heterogeneous regarding diagnostics and treatment. Timely diagnosis is challenging and mainly follows from increased isotope uptake on nuclear examination. Biopsies, autoantibodies and HLA-status are non-contributory, and biochemical inflammation only variably detected. Based on reported data, bisphosphonates and biologicals seem reasonably effective, but due to limitations in design and heterogeneity between studies the precise magnitude of their effect is uncertain. Fundamentally, international consensus seems imperative to advance clinical care for CNO/SCCH.

中文翻译：

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成人胸肋锁区慢性非细菌性骨髓炎 (CNO) 的临床和治疗多样性：一项荟萃分析

目的 慢性非细菌性骨髓炎 (CNO) 是一种罕见的炎症性骨病。影响前胸壁的独特 CNO 亚型被描述为胸肋锁骨肥厚 (SCCH)，主要发生在成人中。关于CNO/SCCH的文献零散，缺乏诊断和治疗共识。方法 系统评价和荟萃分析旨在描述 1) 临床表现和 i2) 应用于成人 CNO/SCCH 患者的治疗方式。未转换的数值数据和双反正弦转换的比例数据被合并到 R-4.0.5 中的随机效应模型中；比例报告为 95% 置信区间 (95%CI)。结果 纳入了 40 项研究，分别包含针对目标 1 和目标 2 的 2030 名患者和 642 名患者的数据。女性倾向（67%，95%CI60-73) 和主要诊断延迟（5 年 95%CI3-7）被记录下来。临床表现包括胸痛 (89%, 95%CI79-96) 和肿胀 (79%, 95%CI62-91)。患者患有掌跖脓疱病（53%，95%CI37-68），关节炎（24%，95%CI 11-39）和痤疮（8%，95%CI4-13）。炎症标志物不一致地升高。自身抗体和 HLA-B27 患病率正常，组织病理学无特异性。同位素摄取增加 (99%, 95% CI96-100) 是一致的成像发现。在多种治疗中，帕米膦酸盐和生物制剂分别产生了 83%、95%CI60-98 和 56%、95%CI26-85 的良好反应。结论 CNO/SCCH 文献证明在诊断和治疗方面存在异质性。及时诊断具有挑战性，主要是由于核检查时同位素摄取增加所致。活检，自身抗体和 HLA 状态是非贡献性的，生化炎症只能可变地检测到。根据报告的数据，双膦酸盐和生物制剂似乎相当有效，但由于设计的局限性和研究之间的异质性，它们的作用的精确程度尚不确定。从根本上说，国际共识似乎势在必行，以推进 CNO/SCCH 的临床护理。