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SAPHO Syndrome — SCE Rheumatology MCQ

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HardSpondyloarthropathySAPHO SyndromeSCE Rheumatology

A 45-year-old man has an 18-month history of recurrent pain and swelling around the manubrium and medial ends of both clavicles. He is afebrile, and repeated blood and joint-aspirate cultures are negative. CT demonstrates osteosclerosis, cortical thickening and hyperostosis centred on the sternoclavicular and first sternocostal joints. Bone scintigraphy shows increased uptake in both sternoclavicular regions and the manubrium. He has recurrent sterile palmoplantar pustules and a previous history of severe nodulocystic acne. What is the most likely unifying diagnosis?

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Correct answer: D — SAPHO syndrome

The diagnosis is **SAPHO syndrome**. The discriminating combination is sterile inflammatory osteoarticular disease of the anterior chest wall, osteosclerosis and hyperostosis, together with palmoplantar pustulosis and severe acne. Bilateral sternoclavicular–manubrial uptake is the characteristic distribution underlying the bone-scan “bull’s head” pattern. Sternoclavicular septic arthritis is less likely because the course is chronic and multifocal, cultures are repeatedly negative, and proliferative bone change predominates. Axial spondyloarthritis may overlap phenotypically but does not adequately explain this characteristic skin–bone constellation. Paget disease can produce bone expansion and sclerosis but not the associated sterile pustulosis and acne. Tietze syndrome causes localised costochondral swelling without osteitis, hyperostosis or the characteristic dermatological manifestations.

Reference: Furer V, Kishimoto M, Tomita T, Elkayam O, Helliwell PS. Pro and contra: is synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) a spondyloarthritis variant? Current Opinion in Rheumatology. 2022;34(4):209–217. https://pubmed.ncbi.nlm.nih.gov/35699334/