Department of Internal Medicine, Division of Endocrinology.
Center for Bone Quality, Leiden University Medical Center, Leiden, The Netherlands.
Rheumatology (Oxford). 2023 Feb 1;62(2):512-522. doi: 10.1093/rheumatology/keac443.
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.
Systematic review and meta-analysis aiming to characterize clinical presentation and 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% CI.
Forty studies were included, containing data on 2030 and 642 patients for aim 1 and 2, respectively. A female predisposition (67%, 95% CI 60, 73) and major diagnostic delay (5 years 95% CI 3, 7) were noted. Clinical presentation included chest pain (89%, 95% CI 79, 96) and swelling (79%, 95% CI 62, 91). Patients suffered from pustulosis palmoplantaris (53%, 95% CI 37, 68), arthritis (24%, 95% CI 11, 39) and acne (8%, 95% CI 4, 13). Inflammatory markers were inconsistently elevated. Autoantibody and HLA-B27 prevalence was normal, and histopathology unspecific. Increased isotope uptake (99%, 95% CI 96, 100) was a consistent imaging finding. Among manifold treatments, pamidronate and biologicals yielded good response in 83%, 95% CI 60, 98 and 56%, 95% CI 26, 85, respectively.
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.
慢性非细菌性骨髓炎（CNO）是一种罕见的炎症性骨病。一种特殊的 CNO 亚型影响前胸壁，被命名为胸骨锁骨骨肥厚（SCCH），主要发生在成年人中。关于 CNO/SCCH 的文献较为分散，缺乏诊断和治疗共识。
本研究旨在对成年 CNO/SCCH 患者的临床表现和治疗方法进行系统评价和荟萃分析。未转换的数值数据和双弧形转换的比例数据在 R-4.0.5 中以随机效应模型进行汇总；比例报告带有 95%置信区间。
共纳入 40 项研究，其中目标 1 包含 2030 例患者的数据，目标 2 包含 642 例患者的数据。研究发现女性发病倾向（67%，95%置信区间 60，73）和主要诊断延迟（5 年 95%置信区间 3，7）。临床表现包括胸痛（89%，95%置信区间 79，96）和肿胀（79%，95%置信区间 62，91）。患者还患有脓疱性掌跖脓疱病（53%，95%置信区间 37，68）、关节炎（24%，95%置信区间 11，39）和痤疮（8%，95%置信区间 4，13）。炎症标志物升高不一致。自身抗体和 HLA-B27 的患病率正常，组织病理学无特异性。核检查中一致存在同位素摄取增加（99%，95%置信区间 96，100）。在多种治疗方法中，帕米膦酸盐和生物制剂的有效率分别为 83%（95%置信区间 60，98）和 56%（95%置信区间 26，85）。
CNO/SCCH 的文献在诊断和治疗方面存在异质性。及时诊断具有挑战性，主要依赖于核检查中同位素摄取的增加。活检、自身抗体和 HLA 状态没有帮助，生化炎症也只是不同程度地被检测到。根据报告的数据，双膦酸盐和生物制剂似乎是有效的，但由于设计的局限性和研究之间的异质性，其确切疗效尚不确定。从根本上讲，国际共识对于提高 CNO/SCCH 的临床护理水平至关重要。
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