SAPHO syndrome
Updates to Article Attributes
The SAPHO syndrome is an acronym that refers to a rare conditionsyndrome that is manifested by a combined occurrence of 2:
- S: synovitis
- A: acne
- P: pustulosis
- H: hyperostosis
- O: osteitis
Epidemiology
It classically tends to present in young to middle-aged adults. Presentation in the paediatric 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:
- sternoclavicular joint: most common location of involvement, with osteitis and hyperostosis
- sacroiliitis
- osteosclerosis of vertebral bodies
- long bones: metaphyseal osteosclerosis and osteolysis 5
Nuclear medicine
Increased uptake at bone scan centred 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 paediatric population) 6.
History and etymology
ChamotChamot et al. initially described it in 1987 3.
-<p>The <strong>SAPHO syndrome</strong> is an acronym that refers to a rare condition that is manifested by a combined occurrence of <sup>2</sup></p><ul>- +<p>The <strong>SAPHO syndrome</strong> is an acronym that refers to a rare <a title="Syndrome" href="/articles/syndrome">syndrome</a> that is manifested by a combined occurrence of <sup>2</sup>:</p><ul>
-<a title="Sternoclavicular joint" href="/articles/sternoclavicular-joint">sternoclavicular joint</a>: most common location of involvement, with <a href="/articles/osteitis">osteitis</a> and <a href="/articles/hyperostosis">hyperostosis</a>- +<a href="/articles/sternoclavicular-joint">sternoclavicular joint</a>: most common location of involvement, with <a href="/articles/osteitis">osteitis</a> and <a href="/articles/hyperostosis">hyperostosis</a>
-</ul><h5>Nuclear medicine</h5><p>Increased uptake at <a href="/articles/bone-scintigraphy-1">b</a><a href="/articles/bone-scan">one scan</a> centred on both sternocostoclavicular joints in the characteristic "bull's head" configuration. </p><h4>Treatment and prognosis</h4><p>Tends to be benign although patients may have recurrent bouts of attacks. Pamidronate therapy has been successfully trialled for symptom relief (especially in the paediatric population) <sup>6</sup>.</p><h4>History and etymology</h4><p><strong>Chamot </strong>et al. initially described it in 1987 <sup>3</sup>.</p>- +</ul><h5>Nuclear medicine</h5><p>Increased uptake at <a href="/articles/bone-scintigraphy-1">b</a><a href="/articles/bone-scintigraphy-1">one scan</a> centred on both sternocostoclavicular joints in the characteristic "bull's head" configuration. </p><h4>Treatment and prognosis</h4><p>Tends to be benign although patients may have recurrent bouts of attacks. Pamidronate therapy has been successfully trialled for symptom relief (especially in the paediatric population) <sup>6</sup>.</p><h4>History and etymology</h4><p>Chamot<strong> </strong>et al. initially described it in 1987 <sup>3</sup>.</p>
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