A 50-year-old woman presented to our tertiary center with chest pain that had been present for 2 years, without weight loss or fever. Physical examination revealed a bony manubrial swelling with prominent vasculature, with oedema of the left hemithorax, and a pustular rash on the foot soles. ESR was 65 mm/h, haemoglobin 7.8 mmol/l, and ALP 69 U/l. Single-photon emission CT demonstrated sclerosis, hyperostosis and increased uptake of the manubrium and upper ribs (Fig. 1, left pane). The pustular rash was diagnosed as pustulosis palmoplantaris, and the total clinical picture was suggestive of chronic non-bacterial osteomyelitis in the context of synovitis, acne, pustulosis, hyperostosis, osteitis syndrome (CNO/SAPHO). Contrast-CT showed obstruction of the left subclavian vein with expansive collaterals (Fig. 1, right pane), presumably resulting from a thrombotic event caused by hyperostotic compression. No malignant lesions were observed. Prophylactic apixaban was started, and the patient was treated with 3-monthly i.v. pamidronate with good clinical effect on bone pain.
Figure 1.
Single-photon emission CT and contrast CT of the anterior chest wall demonstrating CNO/SAPHO complicated by subclavian vein obstruction. Left pane: arrows indicate sclerosis, hyperostosis and increased uptake of the manubrium and upper ribs. Right pane: arrows indicate obstruction of the left subclavian vein with expansive collaterals
CNO/SAPHO mostly manifests as osteitis of the anterior chest wall in adults and often co-occurs with dermatologic or articular inflammation [1]. In long-existing CNO/SAPHO, hyperostosis may cause neurovascular compression, which may trigger thrombosis warranting anticoagulants [2].
Contributor Information
Anne T Leerling, Department of Internal Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands; Center for Bone Quality, Leiden University Medical Center, Leiden, The Netherlands.
Ana Navas Cañete, Center for Bone Quality, Leiden University Medical Center, Leiden, The Netherlands; Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.
Elizabeth M Winter, Department of Internal Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands; Center for Bone Quality, Leiden University Medical Center, Leiden, The Netherlands.
Data availability
Data are available on request.
Funding
No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.
Disclosure statement: The authors have declared no conflicts of interest.
References
- 1. Leerling A, Dekkers O, Appelman-Dijkstra N, Winter E.. Clinical and therapeutic diversity in adult chronic nonbacterial osteomyelitis (CNO) of the sternocostoclavicular region: a meta-analysis. Rheumatology (Oxford) 2023;62:512–22. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2. Godot A, Fakih O, Prati C, Wendling D, Verhoeven F.. SAPHO syndrome and subclavian thrombosis: simple fortuitous association? Eur J Intern Med 2020;72:103–5. [DOI] [PubMed] [Google Scholar]
Associated Data
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Data Availability Statement
Data are available on request.