SAPHO-Syndrom
The SAPHO syndrome is an acronym that refers to a rare syndrome that is manifested by a combined occurrence of :
- 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 pediatric population is not uncommon.
Pathology
SAPHO and related conditions have three stages of progression, which are described as:
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
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 .
Siehe auch:
- Sakroiliitis
- Articulatio sternoclavicularis
- Sklerose des Sternoclaviculargelenks
- Sklerosierung der Klavikula