J Rheum Dis.  2012 Feb;19(1):51-54. 10.4078/jrd.2012.19.1.51.

A Case of Refractory SAPHO Syndrome Treated with Etanercept

• Kim YJ
• Bae SI
• Choi SJ
• Lee YH
• Ji JD
• Song GG

• KMID: 2223121
• DOI: http://doi.org/10.4078/jrd.2012.19.1.51

Abstract

SAPHO syndrome, which has different skin changes and osteoarticular inflammation, is an acronym that stands for synovitis, acne, pustulosis, hyperostosis, and osteitis. Treatment of SAPHO syndrome includes non-steroidal anti-inflammatory drugs (NSAIDs), anti-rheumatic drugs, such as colchicines, corticosteroids and bisphosphonates, and disease-modifying agents. However, the treatment of SAPHO syndrome is controversial because it is a new clinical entity with unclear etiopathogenesis and inadequate clinical studies. We report a case with refractory SAPHO syndrome, which was successfully treated with a tumor necrosis factor (TNF)-alpha blocker.

Keyword

SAPHO syndrome; Treatment; TNF-alpha blocker

MeSH Terms

Acne Vulgaris
Acquired Hyperostosis Syndrome
Adrenal Cortex Hormones
Antirheumatic Agents
Diphosphonates
Hyperostosis
Immunoglobulin G
Inflammation
Osteitis
Receptors, Tumor Necrosis Factor
Skin
Synovitis
Tumor Necrosis Factor-alpha
Etanercept
Adrenal Cortex Hormones
Antirheumatic Agents
Diphosphonates
Immunoglobulin G
Receptors, Tumor Necrosis Factor
Tumor Necrosis Factor-alpha

Figure

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  Figure 1 Acne in different size and shape were seen in his face.

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  Figure 2 Coronal FSE fat saturated T2 weighted image showed irregularity of joint space and subchondral bone marrow edema and enhancement at the sacroiliac joint (arrow). These findings suggest sacroilitis.

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  Figure 3 Technetium-99m bone scan shows increased uptake in the sternocleidoclavicular regions. This is known as "bull's head" or "bull-horn" sign and is characteristic for SAPHO syndrome.

Reference

1. Olivieri I, Padula A, Ciancio G, Salvarani C, Niccoli L, Cantini F. Successful treatment of SAPHO syndrome with infliximab: report of two cases. Ann Rheum Dis. 2002. 61:375–376.

2. Matzaroglou Ch, Velissaris D, Karageorgos A, Marangos M, Panagiotopoulos E, Karanikolas M. SAPHO Syndrome Diagnosis and Treatment: Report of Five Cases and Review of the Literature. Open Orthop J. 2009. 3:100–106.

3. Hayem G, Bouchaud-Chabot A, Benali K, Roux S, Palazzo E, Silbermann-Hoffman O, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum. 1999. 29:159–171.

4. Cotten A, Flipo RM, Mentre A, Delaporte E, Duquesnoy B, Chastanet P. SAPHO syndrome. Radiographics. 1995. 15:1147–1154.

5. Eyigor S, Karapolat H, Adanur H, Kirazli Y. SAPHO syndrome with adrenal deficiency: a case report. Cases J. 2009. 2:6281.

6. Hayem G. Valuable lessons from SAPHO syndrome. Joint Bone Spine. 2007. 74:123–126.

7. Van Doornum S, Barraclough D, McColl G, Wicks I. SAPHO: rare or just not recognized? Semin Arthritis Rheum. 2000. 30:70–77.

8. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A. Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases. Rev Rhum Mal Osteoartic. 1987. 54:187–196.

9. Assmann G, Kueck O, Kirchhoff T, Rosenthal H, Voswinkel J, Pfreundschuh M, et al. Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study. Arthritis Res Ther. 2009. 11:R140.

10. Schwartz BD. Infectious agents, immunity, and rheumatic diseases. Arthritis Rheum. 1990. 33:457–465.

11. Duncan RC, Hay EM, Saklatvala J, Croft PR. Prevalence of radiographic osteoarthritis--it all depends on your point of view. Rheumatology (Oxford). 2006. 45:757–760.

12. Ben Abdelghani K, Dran DG, Gottenberg JE, Morel J, Sibilia J, Combe B. Tumor necrosis factor-alpha blockers in SAPHO syndrome. J Rheumatol. 2010. 37:1699–1704.

13. Vilar-Alejo J, Dehesa L, de la Rosa-del Rey P, Novoa-Medina J, Valerón Almazán P, Santana Medina N, et al. SAPHO syndrome with unusual cutaneous manifestations treated successfully with etanercept. Acta Derm Venereol. 2010. 90:531–532.