SAPHO syndrome

SAPHO syndrome

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SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic and pathologic characteristics. An entity initially known as Chronic recurrent multifocal osteomyelitis (CRMO) was first described in 1972. Subsequently in 1978 several cases of CRMO were associated with blisters on the palms and soles (palmoplantar pustulosis). Since then, a number of associations between skin conditions and osteoarticular disorders have been reported under a variety of names, including sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, and acne-associated spondyloarthropathy. The term SAPHO was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.

Definition

*Synovitis
*Acne -- commonly involving the face and upper back.
*Pustulosis -- usually involving the palms of the hands and/or soles of the feet (palmo-plantar pustulosis).
*Hyperostosis
*Osteitis

Radiologic Findings

Anterior chest wall (most common site, 65-90% of patients): Hyperostosis, sclerosis and bone hypertrophy especially involving the sternoclavicular joint, often with a soft tissue component.

Spine (33% of patients): Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement.

Long bones (30% of patients): usually metadiaphyseal and located in the distal femur and proximal tibia. It looks like chronic osteomyelitis but will not have a sequestrum or abscess.

Flat bones (10% of patients): mandible and ilium.

Peripheral arthritis has been reported in 92% of cases of SAPHO as well.

In children the SAPHO syndrome is most likely to affect the metaphysis of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.

Treatment

Treatment of patients with SAPHO syndrome is based on clinical symptoms. Generally, treatment involves Non-steroidal anti-inflammatory drugs and corticosteroid medications (either in the form of topical creams, tablets, or by injection into the involved area). Topical cold applications may also help in affected areas. If unsuccessful, both sulfasalazine and methotrexate have been tried with mixed results.

References

* [http://uwmsk.org/moodle/mod/resource/view.php?id=407 SAPHO Syndrome] by Mike Chang, M.D., University of Washington Department of Radiology
* [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12719925&dopt=Abstract Skeletal Radiology] . 2003 Jun;32(6):311-27.
* [http://www.medicinenet.com/sapho_syndrome/article.htm MedicineNet.com]
* [http://en.wikipedia.org/wiki/Chronic_recurrent_multifocal_osteomyelitis]

Treamtment with anti-TNF have been tried in few paitents with limited success.


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