SAPHO syndrome
Disclosures
- updated 17 Aug 2022:
Nothing to disclose
Updates to Article Attributes
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The SAPHO syndrome is an acronym that refers to a rare syndrome 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. There may be slightly higher female predilection 8.
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
Aetiology
Many causes have been suggested with infectious aetiology considerably favoured. Propionibacterium acnes, an anaerobic saprophyte found in human skin, has been isolated from the biopsy specimens of bone and synovium 8.
Serology
HLAB27: may be positive in around 30% of patients 8
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
osteosclerosis of vertebral bodies
long bones: metaphyseal osteosclerosis and osteolysis 5
Nuclear medicine
Increased uptake at bonebone 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 trialledtrialed for symptom relief (especially in the paediatric population) 6.
Differential diagnosis
For bony features in children consider
History and etymology
Chamotet al. initially described it in 1987 3.
-<p>The <strong>SAPHO syndrome</strong> is an acronym that refers to a rare <a href="/articles/syndrome">syndrome</a> that is manifested by a combined occurrence of <sup>2</sup>:</p><ul>-<li>-<strong>S: </strong><a href="/articles/synovitis">synovitis</a>-</li>-<li>-<strong>A: </strong><a href="/articles/acne">acne</a>-</li>-<li>-<strong>P: </strong><a href="/articles/pustulosis">pustulosis </a>-</li>-<li>-<strong>H: </strong><a href="/articles/hyperostosis">hyperostosis</a>-</li>-<li>-<strong>O: </strong><a href="/articles/osteitis">osteitis</a>-</li>-</ul><h4>Epidemiology</h4><p>It classically tends to present in young to middle-aged adults. Presentation in the paediatric population is not uncommon. There may be slightly higher female predilection <sup>8</sup>.</p><h4>Pathology</h4><p>SAPHO and related conditions have three stages of progression, which are described as:</p><ol>-<li>-<a href="/articles/costoclavicular-ligament">costoclavicular ligament</a> ossification</li>-<li>arthropathy of the <a href="/articles/sternoclavicular-joint">sternoclavicular joint</a>, osteitis of the medial end of the clavicle, first rib and sternum as well as hypertrophy of the costal cartilages</li>-<li>osteitis, <a href="/articles/hyperostosis">hyperostosis</a> and hypertrophy of the medial ends of the clavicles, sternum and upper ribs and ultimately <a href="/articles/ankylosis">ankylosis</a>-</li>-</ol><h5>Aetiology</h5><p>Many causes have been suggested with infectious aetiology considerably favoured. <em>Propionibacterium acnes</em>, an anaerobic saprophyte found in human skin has been isolated from the biopsy specimens of bone and synovium <sup>8</sup></p><h5>Serology</h5><ul><li>HLAB27: may be positive in around 30% of patients<sup> 8</sup>-</li></ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Plain radiograph is non-specific, but SAPHO syndrome can be suspected if present with the other clinical findings:</p><ul>-<li>-<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>-</li>-<li><a href="/articles/sacroiliitis-differential">sacroiliitis</a></li>-<li>-<a href="/articles/osteosclerosis">osteosclerosis</a> of vertebral bodies</li>-<li>long bones: metaphyseal osteosclerosis and <a href="/articles/osteolysis">osteolysis</a> <sup>5</sup>-</li>-</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>Differential diagnosis</h4><p>For bony features in children consider </p><ul><li><a title="Chronic recurrent multifocal osteomyelitis" href="/articles/chronic-recurrent-multifocal-osteomyelitis">chronic recurrent multifocal osteomyelitis (CRMO) </a></li></ul><h4>History and etymology</h4><p>Chamot<strong> </strong>et al. initially described it in 1987 <sup>3</sup>.</p>- +<p>The <strong>SAPHO syndrome</strong> is an acronym that refers to a rare <a href="/articles/syndrome">syndrome</a> that is manifested by a combined occurrence of <sup>2</sup>:</p><ul>
- +<li><p><strong>S: </strong><a href="/articles/synovitis">synovitis</a></p></li>
- +<li><p><strong>A: </strong><a href="/articles/acne">acne</a></p></li>
- +<li><p><strong>P: </strong><a href="/articles/pustulosis">pustulosis</a></p></li>
- +<li><p><strong>H: </strong><a href="/articles/hyperostosis">hyperostosis</a></p></li>
- +<li><p><strong>O: </strong><a href="/articles/osteitis">osteitis</a></p></li>
- +</ul><h4>Epidemiology</h4><p>It classically tends to present in young to middle-aged adults. Presentation in the paediatric population is not uncommon. There may be slightly higher female predilection <sup>8</sup>.</p><h4>Pathology</h4><p>SAPHO and related conditions have three stages of progression, which are described as:</p><ol>
- +<li><p><a href="/articles/costoclavicular-ligament">costoclavicular ligament</a> ossification</p></li>
- +<li><p>arthropathy of the <a href="/articles/sternoclavicular-joint">sternoclavicular joint</a>, osteitis of the medial end of the clavicle, first rib and sternum as well as hypertrophy of the costal cartilages</p></li>
- +<li><p>osteitis, <a href="/articles/hyperostosis">hyperostosis</a> and hypertrophy of the medial ends of the clavicles, sternum and upper ribs and ultimately <a href="/articles/ankylosis">ankylosis</a></p></li>
- +</ol><h5>Aetiology</h5><p>Many causes have been suggested with infectious aetiology considerably favoured. <em>Propionibacterium acnes</em>, an anaerobic saprophyte found in human skin, has been isolated from the biopsy specimens of bone and synovium <sup>8</sup>.</p><h5>Serology</h5><ul><li><p>HLAB27: may be positive in around 30% of patients<sup> 8</sup></p></li></ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Plain radiograph is non-specific, but SAPHO syndrome can be suspected if present with the other clinical findings:</p><ul>
- +<li><p><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></p></li>
- +<li><p><a href="/articles/sacroiliitis-differential">sacroiliitis</a></p></li>
- +<li><p><a href="/articles/osteosclerosis">osteosclerosis</a> of vertebral bodies</p></li>
- +<li><p>long bones: metaphyseal osteosclerosis and <a href="/articles/osteolysis">osteolysis</a> <sup>5</sup></p></li>
- +</ul><h5>Nuclear medicine</h5><p>Increased uptake at <a href="/articles/bone-scintigraphy-1">bone 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 trialed for symptom relief (especially in the paediatric population) <sup>6</sup>.</p><h4>Differential diagnosis</h4><p>For bony features in children consider</p><ul><li><p><a href="/articles/chronic-recurrent-multifocal-osteomyelitis" title="Chronic recurrent multifocal osteomyelitis">chronic recurrent multifocal osteomyelitis (CRMO) </a></p></li></ul><h4>History and etymology</h4><p>Chamot<strong> </strong>et al. initially described it in 1987 <sup>3</sup>.</p>
Systems changed:
- Paediatrics
Images Changes:
Image 5 CT (bone window) ( update )
Caption
was added:
Case 4
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