SAPHO syndrome

Last revised by Henry Knipe on 3 Nov 2023

The SAPHO syndrome is an acronym that refers to a rare syndrome that is manifested by a combined occurrence of 2:

SAPHO classically tends to present in young to middle-aged adults. Presentation in the pediatric population is not uncommon. There may be slightly higher female predilection 8.

SAPHO and related conditions have three stages of progression, which are described as:

  1. costoclavicular ligament ossification

  2. arthropathy of the sternoclavicular joint, osteitis of the medial end of the clavicle, first rib and sternum as well as hypertrophy of the costal cartilages

  3. osteitis, hyperostosis and hypertrophy of the medial ends of the clavicles, sternum and upper ribs and ultimately ankylosis

Many causes have been suggested with infectious etiology considerably favored. Propionibacterium acnes, an anaerobic saprophyte found in human skin, has been isolated from the biopsy specimens of bone and synovium 8.

  • HLAB27: may be positive in around 30% of patients 8

Plain radiograph is non-specific, but SAPHO syndrome can be suspected if present with the other clinical findings:

Increased uptake at bone scan centered on both sternocostoclavicular joints in the characteristic "bull's head" configuration. 

Tends to be benign although patients may have recurrent bouts of attacks. Pamidronate therapy has been successfully trialed for symptom relief (especially in the pediatric population) 6.

For bony features in children consider

Chamot et al. initially described it in 1987 3.

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