Mandibular Involvement in Recurrent Multifocal Osteomyelitis Associated with SAPHO Syndrome

Document Type : Case Report

Authors

1 Department of Otolaryngology Head and Neck, State University of Campinas, Faculty of Medical Sciences, Campinas, Sao Paulo, Brazil.

2 Otorhinolaryngologist, Coordinator of Ambulatory Surgery and Maxillofacial Traumatology, State University of Campinas, Faculty of Medical Sciences, Sao Paulo, Brazil.

Abstract

Introduction:
SAPHO syndrome is defined as the association of a group of rare sterile osteoarticular disorders and inflammatory skin diseases whose etiology, although not yet determined, probably involves genetic, immunological and infectious mechanisms. The recurrent multifocal osteomyelitis, an inflammatory disease, can be associated with this syndrome even as a single event.
 Case Report:
A case of a young female patient, with a definite diagnosis of SAPHO and an inflammatory mandibular atypical disease for which therapeutic options with immunosuppressants were being used, is reported. The adverse evolution of the clinical conditions led to the hypothesis that the patient suffered from associated mandibular odontogenic bacterial osteomyelitis. The extraction of all teeth was recommended. After our evaluation, we recommended a conservative treatment, and after 2 months of treatment with an endovenous antibiotic, the patient showed improvement of clinical and laboratory results.
 Conclusion:
Early diagnosis is necessary to avoid successive and unnecessary tooth loss in the treatment of chronic osteomyelitis mandibular. 

Keywords

Main Subjects


1. Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME. The SAPHO syndrome. Semin Arthritis Rheum. 2012 Dec;42(3):254-65.
2. Mochizuki Y, Omura K, Hirai H, Kugimoto T, Osako T, Taguchi T. Chronic mandibular osteomyelitis with suspected underlying synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: a case report. J Inflamm Res. 2012; 5:
29-35.
3. Burgemeister LT, Baeten DL, Tas SW. Biologics for rare inflammatory diseases: TNF blockade in the SA PHO syndrome. Neth J Med. 2012 Dec; 70 (10): 444-9.
4. Kuijpers SC, de Jong E, Hamdy NA, van Merkesteyn JP. Initial results of the treatment of diffuse sclerosing osteomyelitis of the mandible with bisphosphonates. J Craniomaxillofac Surg. 2011 Jan; 39(1):65-8.
5. Aubry-Rozier B, Basch A, Dudler J. Diffuse sclerosing osteomyelitis of the mandible and SAPHO. Rev Med Interne. 2012 Jun;33(6):e34-7.
6. Suei Y, Taguchi A, Tanimoto K. Diagnosis and classification of mandibular osteomyelitis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Aug;100(2):207-14.
7. Magrey M, Khan MA. New insights into synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Curr Rheumatol Rep. 2009 Oct; 11(5):329-33.
8. Karadag-Saygi E, Gunduz OH, Gumrukcu G, Akyuz G. SAPHO syndrome:
misdiagnosed and operated. Acta Reumatol Port. 2008 Oct-Dec;33(4):460-3. 
9. Assmann G, Simon P. The SAPHO syndrome - are microbes involved? Best Pract Res Clin Rheumatol. 2011 Jun;25(3):423-34.
10. Fleuridas G, Teysseres N, Ragot JP, Chikhani L, Favre-Dauvergne E. Diffuse sclerosing osteomyelitis of the mandible and SAPHO syndrome. Rev Stomatol Chir Maxillofac. 2002 Apr;103(2):96-104.
11. Zemann W, Pau M, Feichtinger M, Ferra-Matschy B, Kaercher H. SAPHO syndrome with affection of the mandible: diagnosis, treatment, and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011 Feb;111(2):190-5.
12. Olivieri I, Padula A, Palazzi C. Pharmacological management of SAPHO syndrome.  Expert Opin Investig Drugs. 2006 Oct; 15(10):1229-33
13. Ben Abdelghani K, Dran DG, Gottenberg JE, Morel J, Sibilia J, Combe B. Tumor necrosis factor-alpha blockers in SAPHO syndrome. Rheumatol. 2010 Aug 1;37(8):1699-704.
14. De Souza A, Solomon GE, Strober BE. SAPHO syndrome associated with hidradenitis suppurativa successfully treated with infliximab and methotrexate. Bull NYU Hosp Jt Dis. 2011; 69(2): 185-7.
15. Garcovich S, Amelia R, Magarelli N, Valenza V, Amerio P. Long-term treatment of severe SAPHO syndrome with adalimumab: case report and a review of the literature. Am J Clin Dermatol. 2012 Feb 1;13(1):55-9. 
16. Hatano H, Shigeishi H, Higashikawa K, Shimasue H, Nishi H, Oiwa H, et al. A case of SAPHO syndrome with diffuse sclerosing osteomyelitis of the mandible treated successfully with prednisolone and bisphosphonate. J Oral Maxillofac Surg. 2012 Mar; 70(3):626-31.
17. Yamazaki Y, Satoh C, Ishikawa M, Notani K, Nomura K, Kitagawa Y. Remarkable response of juvenile diffuse sclerosing osteomyelitis of mandible to pamidronate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Jul;104(1):67-71.
18. Baltensperger M, Grätz K, Bruder E, Lebeda R, Makek M, Eyrich G. Is primary chronic osteomyelitis a uniform disease? Proposal of a classification based on a retrospective analysis of patients treated in the past 30 years. J Craniomaxillofac Surg. 2004 Feb;32(1):43-50.
19. Lapi F, Cipriani F, Caputi AP, Corrao G, Vaccheri A, Sturkenboom MC, et al. Bisphosphonates Efficacy-Safety Tradeoff (BEST) study group. Assessing the risk of osteonecrosis of the jaw due to bisphosphonate therapy in the secondary prevention of osteoporotic fractures. Osteoporos Int. 2013 Feb;24(2):697-705.
20. Weinstein RS. Glucocorticoid-induced osteoporosis and osteonecrosis. Endocrinol Metab Clin North Am. 2012 Sep;41(3):595-611.