Clinical and therapeutic diversity in adult chronic nonbacterial osteomyelitis (CNO) of the sternocostoclavicular region: a meta-analysis

Abstract

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.

Hyperostosis of the Clavicle as a Cardinal Symptom for Sapho Syndrome – Presentation and Discussion of a Clinical Case

“…Possibly due to different inflammatory pathways involved, biologics in particular show different effects on bone, joint and skin lesions in SAPHO [20,32], whereby the exact extent of their effect is uncertain [33]. The tumour necrosis factor blockers, JAK inhibitors, and interleukin-1 and -17 inhibitors, which are selected and adapted according to the clinical picture, are of greatest importance [32].…”

Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome with mandibular involvement: Would surgical operation help?

“…The first‐line clinical medications for SAPHO syndrome are mainly NSAIDs and corticosteroids. A systematic review on chronic nonbacterial osteomyelitis (CNO) and sternocostoclavicular hyperostosis (sCCH) showed that only 9 patients responded well to surgery in 285 patients 8 . A report of 2 cases of SAPHO syndrome involving the left mandible showed success after combined treatment with corticosteroids 2 .…”

Osteoarthropathy of the front thorax

“…Некоторые авторы, правда, считают, что отличительной чертой синдрома SAPHO является относительно частое развитие артрита периферических суставов, что не свойственно другим формам ХНО. По данным метаанализа исследований, в которые включены 642 взрослых пациента с ХНО (при наличии ОАПГК), периферический артрит имел место в 24% случаев [9]. Сообщается в основном о моноартрите или олигоартрите различных суставов, в том числе мелких суставов кистей и стоп, иногда даже дистальных межфаланговых.…”