SAPHO syndrome — SCE Rheumatology MCQ

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ModerateSpondyloarthropathiesSAPHO syndromeSCE Rheumatology

A 37-year-old woman has anterior chest wall pain, pustular lesions on the palms and recurrent sterile osteitis of the clavicle. Bone scan shows increased uptake at the sternoclavicular region. Cultures from previous biopsy were negative and inflammatory markers fluctuate. What is the most likely diagnosis?

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Correct answer: C — SAPHO syndrome

Explanation lettering: C = shown as A · A = shown as B · E = shown as C · B = shown as D · D = shown as E

SAPHO syndrome is best because sterile osteitis/hyperostosis of the anterior chest wall with palmoplantar pustulosis is typical of SAPHO syndrome. A is less suitable because Paget disease causes focal high bone turnover but not pustulosis and sterile osteitis pattern; B is less suitable because septic arthritis would have positive cultures or acute infection features; C is less suitable because Tietze syndrome does not cause osteitis on bone scan or pustular skin disease; D is less suitable because metastatic cancer must be excluded but the recurrent sterile inflammatory pattern supports SAPHO. Clinical pearl: skin and bone findings together are the diagnostic clue in SAPHO.

Reference: Oxford Textbook of Rheumatology