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SAPHO syndrome
Epidemiology:
- bimodal distribution: can manifest in childhood or as an aldult
Pathology:
- inflammatory bone disease with or without associated skin disease
Clinical manifestations:
- Synovitis
- Acne - commonly involving the face, neck & upper back
- Palmoplantar pustulosis
- Hyperostosis that affects anterior chest wall & sternoclavicular joints
- Osteitis
other
- sterile osteomyeltitis
- serositis or enthesitis
- hydradenitis supprativa
- pustular psoriasis
Diagnostic criteria:
- no consensus
- bone or joint symptoms + palmoplantar pustulosis
- quadriceps enthesitis is a minor criterion
Radiology:
- X-rays
- anterior chest wall
- hyperostosis
- sclerosis
- bone hypertrophy
- sternoclavicular joint, often involved
- thoracic spine
- spondylodiscitis
- osteosclerosis
- paravertebral ossifications
- pelvis: sacroiliac joint involvement
- long bones: distal femur, proximal tibia
- peripheral joints, arthritis in 92% of cases
- in children
- metaphysis of long bones in the legs (tibia, femur, fibula)
- clavicle
- spine
Management:
- bisphosphonates may be useful
- treatment as psoratic arthritis may be useful
- antibiotics in treatment of acne may be useful
General
syndrome
References
- Wikipedia: SAPHO syndrome
http://en.wikipedia.org/wiki/SAPHO_syndrome
- NEJM Knowledge+ Complex Medical Care
- Li C, Zuo Y, Wu N et al
Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre
study of a cohort of 164 patients.
Rheumatology (Oxford). 2016 Jun;55(6):1023-30.
PMID: 26917545
- Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME.
The SAPHO syndrome.
Semin Arthritis Rheum. 2012 Dec;42(3):254-65.
PMID: 23153960 Free article.
- Zimmermann P, Curtis N.
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome -
A challenging diagnosis not to be missed.
J Infect. 2016 Jul 5;72 Suppl:S106-14.
PMID: 27263075