SAPHO-Syndrom

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

Epidemiology

It classically tends to present in young to middle-aged adults. Presentation in the pediatric population is not uncommon.

Pathology

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

  • costoclavicular ligament ossification
  • 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
  • osteitis, hyperostosis and hypertrophy of the medial ends of the clavicles, sternum and upper ribs and ultimately ankylosis
  • Radiographic features

    Plain radiograph

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

    Nuclear medicine

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

    Treatment and prognosis

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

    History and etymology

    Chamot et al. initially described it in 1987 .

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