Medicine:SAPHO syndrome
| SAPHO syndrome | |
|---|---|
| Other names | Synovitis-acne-pustulosis-hyperostosis-osteitis syndrome |
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 was first described in 1972.[1] Subsequently, in 1978,[2] several cases of 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 (an acronym for synovitis, acne, pustulosis, hyperostosis, osteitis) was coined in 1987[3] to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.
Diagnosis
Radiologic findings
- Anterior chest wall (most common site, 65–90% of patients):[citation needed] Hyperostosis, sclerosis and bone hypertrophy especially involving the sternoclavicular joint, often with a soft tissue component.
- Spine (33% of patients):[citation needed] Segmental, usually involving the thoracic spine. The four main presentations include spondylodiscitis, osteosclerosis, paravertebral ossifications, and sacroiliac joint involvement.
- Long bones (30% of patients):[citation needed] 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):[citation needed] mandible and ilium.
Peripheral arthritis has been reported in 92% of cases of SAPHO as well.[citation needed]
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
Bisphosphonate therapy has been suggested as a first-line therapeutic option in many case reports and series.[4][5][6]
Treatment with tumor necrosis factor alpha antagonists (TNF inhibitors) has been tried in a few patients with limited success.[7] Other drugs that are used in psoriatic arthritis, to which SAPHO syndrome is closely related, have also been used in this condition. They include NSAIDs, corticosteroids, sulfasalazine, methotrexate, ciclosporin and leflunomide.[8]
Some patients have responded to antibiotics. The rationale for their use is that Cutibacterium acnes, a bacterium known for its role in acne, has been isolated from bone biopsies of SAPHO patients.[7][9]
See also
- PAPA syndrome
- Psoriatic arthritis
- List of cutaneous conditions
References
- ↑ "Subacute and chronic "symmetrical" osteomyelitis". Annals of Radiology 15 (15): 329–42. 1972. PMID 4403064.
- ↑ "Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantaris". Journal of Pediatrics 93 (2): 227–31. 1978. doi:10.1016/S0022-3476(78)80501-0. PMID 671154.
- ↑ "Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases". Revue du Rhumatisme et des Maladies Osteo-Articulaires 54 (3): 187–96. 1987. PMID 2954204.
- ↑ "Successful treatment of SAPHO syndrome with an oral bisphosphonate". Rheumatology International 29 (6): 713–5. November 2008. doi:10.1007/s00296-008-0760-z. PMID 18998139.
- ↑ "Pamidronate in the treatment of childhood SAPHO syndrome". Rheumatology (Oxford) 43 (10): 1246–51. October 2004. doi:10.1093/rheumatology/keh295. PMID 15238641.
- ↑ "SAPHO syndrome treated with pamidronate: an open-label study of 10 patients". Rheumatology (Oxford) 43 (5): 658–61. May 2004. doi:10.1093/rheumatology/keh149. PMID 14983108.
- ↑ 7.0 7.1 "Pharmacological management of SAPHO syndrome". Expert Opinion on Investigational Drugs 15 (10): 1229–33. October 2006. doi:10.1517/13543784.15.10.1229. PMID 16989598.
- ↑ "Successful treatment of SAPHO syndrome with leflunomide. Report of two cases". Clinical and Experimental Rheumatology 23 (5): 731. 2005. PMID 16173265.
- ↑ "Propionibacterium acnes and SAPHO syndrome: a case report and literature review". Clinical and Experimental Rheumatology 25 (3): 457–60. 2007. PMID 17631745. http://www.clinexprheumatol.org/pubmed/find-pii.asp?pii=17631745.
Further reading
- SAPHO Syndrome by Mike Chang, M.D., University of Washington Department of Radiology
- "SAPHO: syndrome or concept? Imaging findings". Skeletal Radiol. 32 (6): 311–27. June 2003. doi:10.1007/s00256-003-0629-x. PMID 12719925.
External links
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