SAPHO syndrome

SAPHO syndrome
Classification and external resources
DiseasesDB	30718

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.^[1] Subsequently, in 1978,^[2] 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 (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.

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

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) have been tried in 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 Propionibacterium 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

1. Giedion A, Holthusen W, Masel LF, Vischer D (1972). "Subacute and chronic "symmmetrical" osteomyelitis.". Annals of Radiology (15): 329–42.
2. Bjorksten B, Gustavson KH, Eriksson B, et al. (1978). "Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantaris". Journal of Pediatrics (93): 227–31.
3. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A (1987). "Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases". Rev Rhum Mal Osteoartic. 54 (3): 187–96. PMID 2954204.
4. Ichikawa J, Sato E, Haro H, Ando T, Maekawa S, Hamada Y (November 2008). "Successful treatment of SAPHO syndrome with an oral bisphosphonate". Rheumatol. Int. 29 (6): 713–5. doi:10.1007/s00296-008-0760-z. PMID 18998139.
5. Kerrison C, Davidson JE, Cleary AG, Beresford MW (October 2004). "Pamidronate in the treatment of childhood SAPHO syndrome". Rheumatology (Oxford) 43 (10): 1246–51. doi:10.1093/rheumatology/keh295. PMID 15238641.
6. Amital H, Applbaum YH, Aamar S, Daniel N, Rubinow A (May 2004). "SAPHO syndrome treated with pamidronate: an open-label study of 10 patients". Rheumatology (Oxford) 43 (5): 658–61. doi:10.1093/rheumatology/keh149. PMID 14983108.
7. Olivieri I, Padula A, Palazzi C (October 2006). "Pharmacological management of SAPHO syndrome". Expert Opin Investig Drugs 15 (10): 1229–33. doi:10.1517/13543784.15.10.1229. PMID 16989598.
8. Scarpato S, Tirri E (2005). "Successful treatment of SAPHO syndrome with leflunomide. Report of two cases". Clin. Exp. Rheumatol. 23 (5): 731. PMID 16173265.
9. Colina M, Lo Monaco A, Khodeir M, Trotta F (2007). "Propionibacterium acnes and SAPHO syndrome: a case report and literature review". Clin. Exp. Rheumatol. 25 (3): 457–60. PMID 17631745.

• SAPHO Syndrome by Mike Chang, M.D., University of Washington Department of Radiology
• Earwaker JW, Cotten A (June 2003). "SAPHO: syndrome or concept? Imaging findings". Skeletal Radiol. 32 (6): 311–27. doi:10.1007/s00256-003-0629-x. PMID 12719925.
• MedicineNet.com