SAPHO syndrome
Updates to Article Attributes
Body
was changed:
The SAPHO syndrome is an acronym that refers to a rare condition that is manifested by a combined occurence 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.
Classification
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 clavicle, 1st 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; ultimately ankylosis
Radiographic features
Plain film
Non specific but can be suspected if present with the other clinical findings
-
sternoscalvicularsternoclavicular joint -commonest involvementmost common location of involvement, with osteitis and hyperostosisoccuring in this location sacroileitissacroilitis- osteosclerosis of vertebral bodies
- long bones - metaphyseal osteosclerosis and osteolysis 5
Treatment and prognosis
Generally 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)
Etymology
It was initially described by Chamot et al. in 1987 3
-<li>sternoscalvicular joint - commonest involvement with <a href="/articles/osteitis">osteitis</a> and <a href="/articles/hyperostosis">hyperostosis</a> occuring in this location</li>-<li><a href="/articles/sacroileitis">sacroileitis</a></li>- +<li>sternoclavicular joint - most common location of involvement, with <a href="/articles/osteitis">osteitis</a> and <a href="/articles/hyperostosis">hyperostosis</a>
- +</li>
- +<li><a href="/articles/sacroileitis">sacroilitis</a></li>
Unable to process the form. Check for errors and try again.