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The SAPHO syndrome revisited with an emphasis on spinal manifestations

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Abstract

The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis.

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References

  1. Windom RE, Sandford JP, Ziff M. Acne conglobata and arthritis. Arthritis Rheum. 1961;4:632–5.

    CAS  PubMed  Google Scholar 

  2. Kato T, Kambara H, Hoshi E. Case of bilateral clavicular osteomyelitis with palmar and plantar pustulosis. Seikei Geka. 1968;19:590–3.

    CAS  PubMed  Google Scholar 

  3. Giedion A, Holthusen W, Masel LF, et al. Subacute and chronic “symmetrical” osteomyelitis. Ann Radiol (Paris). 1972;15:329–42.

    CAS  Google Scholar 

  4. Köhler H, Uehlinger E, Kutzner J, et al. Sterno-costo-clavicular hyperostosis: a hitherto undescribed entity (author’s translation). Dtsch Med Wochenschr. 1975;100:1519–23.

    PubMed  Google Scholar 

  5. Björkstén B, Gustavson KH, Eriksson B, et al. Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantaris. J Pediatr. 1978;93:227–31.

    PubMed  Google Scholar 

  6. Bergdahl K, Björkstén B, Gustavson KH, et al. Pustulosis palmoplantaris and its relation to chronic recurrent multifocal osteomyelitis. Dermatologica. 1979;159:37–45.

    CAS  PubMed  Google Scholar 

  7. Sonozaki H, Azuma A, Okai K, et al. Clinical features of 22 cases with “inter-sterno-costo-clavicular ossification”. A new rheumatic syndrome. Arch Orthop Trauma Surg. 1979;95:13–22.

    CAS  PubMed  Google Scholar 

  8. Sonozaki H, Mitsui H, Miyanaga Y, et al. Clinical features of 53 cases with pustulotic arthro-osteitis. Ann Rheum Dis. 1981;40:547–53.

    CAS  PubMed Central  PubMed  Google Scholar 

  9. Dihlmann W, Dihlmann SW. Acquired hyperostosis syndrome: spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases. Clin Rheumatol. 1991;10:250–63.

    CAS  PubMed  Google Scholar 

  10. Lindor NM, Arsenault TM, Solomon H, Seidman CE, McEvoy MT. A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA Syndrome. Mayo Clin Proc. 1997;72:611–5.

    CAS  PubMed  Google Scholar 

  11. Tlougan BE, Podjase JO, O’Haver J, et al. Chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome with associated neutrophilic dermatoses: a report of seven cases and review of the literature. Pediatr Dermatol. 2009;26(5):497–505.

    PubMed  Google Scholar 

  12. Siau K, Laversuch CJ. SAPHO syndrome in an adult with ulcerative colitis responsive to intravenous pamidronate: a case report and review of the literature. Rheumatol Int. 2010;30:1085–8.

    PubMed  Google Scholar 

  13. Chamot AM, Benhamou CL, Kahn MF, et al. Acne–pustulosis–hyperostosis–osteitis syndrome. Results of a national survey. 85 cases. Rev Rhum Mal Osteoartic. 1987;54:187–96.

    CAS  PubMed  Google Scholar 

  14. Boutin RD, Resnick D. The SAPHO syndrome: an evolving concept for unifying several idiopathic disorders of bone and skin. AJR Am J Roentgenol. 1998;170(3):585–91.

    CAS  PubMed  Google Scholar 

  15. Depasquale R, Kumer N, Lalam RK, et al. SAPHO: what radiologists should know. Clin Radiol. 2012;67:195–206.

    CAS  PubMed  Google Scholar 

  16. Assmann G, Simon P. The SAPHO syndrome—are microbes involved? Best Pract Res Clin Rheumatol. 2011;25(3):423–37.

    CAS  PubMed  Google Scholar 

  17. Assmann G, Kueck O, Kirchhoff T, et al. Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study. Arthritis Res Ther. 2009;11:R140.

    PubMed Central  PubMed  Google Scholar 

  18. Govoni M, Colina M, Massara A, Trotta F. SAPHO syndrome and infections. Autoimmun Rev. 2009;8:256–9.

    CAS  PubMed  Google Scholar 

  19. Colina M, La Corte R, Trotta F. Sustained remission of SAPHO syndrome with pamidronate: a follow-up of fourteen cases and review of the literature. Clin Exp Rheumatol. 2009;27:112–5.

    CAS  PubMed  Google Scholar 

  20. Edlund E, Johnsson U, Lidgren L, et al. Palmoplantar pustulosis and sternocostoclavicular arthro-osteitis. Ann Rheum Dis. 1988;47:809–15.

    CAS  PubMed Central  PubMed  Google Scholar 

  21. Trimble BS, Evers CJ, Ballaron SA, Young JM. Intraarticular injection of Propionibacterium acnes causes an erosive arthritis in rats. Agents Actions. 1987;21:281–3.

    CAS  PubMed  Google Scholar 

  22. Colina M, Lo Monaco A, Khodeir M, Trotta F. Propionibacterium acnes and SAPHO syndrome: a case report and literature review. Clin Exp Rheumatol. 2007;25:457–60.

    CAS  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  24. Amital H, Govoni M, Maya R, et al. Role of infectious agents in systemic rheumatic diseases. Clin Exp Rheumatol. 2008;26(1 Suppl 48):S27–32.

    CAS  PubMed  Google Scholar 

  25. Rozin AP. From molecular mimicry to cross-reactivity or pathogen expansion? A hypothesis. Clin Rheumatol. 2007;26:285–8.

    PubMed  Google Scholar 

  26. Rozin AP. SAPHO syndrome: is a range of pathogen-associated rheumatic diseases extended? Arthritis Res Ther. 2009;11(6):131.

    PubMed Central  PubMed  Google Scholar 

  27. Barton GM, Medzhitov R. Toll-like receptor signaling pathways. Science. 2003;300(5625):1524–5.

    CAS  PubMed  Google Scholar 

  28. Kalis C, Gumenscheimer M, Freudenberg N, et al. Requirement of TLR9 in the immunomodulatory activity of P. acnes. J Immunol. 2005;174:4295–300.

    CAS  PubMed  Google Scholar 

  29. Hurtado-Nedelec M, Chollet-Martin S, Chapeton D, Hugot JP, Hayem G, Gérard B. Genetic susceptibility factors in a cohort of 38 patients with SAPHO syndrome: a study of PSTPIP2, NOD2, and LPIN2 genes. J Rheumatol. 2010;37:401–9.

    CAS  PubMed  Google Scholar 

  30. Wagner AD, Andresen J, Jendro MC, Hulsemann JL, Zeidler H. Sustained response to tumor necrosis factor alpha-blocking agents in two patients with SAPHO syndrome. Arthritis Rheum. 2002;46:1965–8.

    PubMed  Google Scholar 

  31. Carpenter E, Jackson MA, Friesen CA, Scarbrough M, Roberts CC. Crohn’s-associated chronic recurrent multifocal osteomyelitis responsive to infliximab. J Pediatr. 2004;144:541–4.

    PubMed  Google Scholar 

  32. 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;61:375–6.

    CAS  PubMed Central  PubMed  Google Scholar 

  33. Jappe U, Boit R, Farrar MD, Ingham E, Sandoe J, Holland KT. Evidence for diversity with Propionibacterium acnes: a comparison of the T cell stimulatory activity of isolates from inflammatory acne, endocarditis and the laboratory. J Eur Acad Dermatol Venereol. 2004;18:450–4.

    CAS  PubMed  Google Scholar 

  34. Jansson A, Renner ED, Ramser J, et al. Classification of non-bacterial osteitis: retrospective study of clinical, immunological and genetic aspects in 89 patients. Rheumatology (Oxford). 2007;46:154–60.

    CAS  Google Scholar 

  35. Ferguson PJ, Chen S, Tayeh MK, et al. Homozygous mutations in LPIN2 are responsible for the syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia (Majeed syndrome). J Med Genet. 2005;42:551–7.

    CAS  PubMed Central  PubMed  Google Scholar 

  36. El-Shanti HI, Ferguson PJ. Chronic recurrent multifocal osteomyelitis: a concise review and genetic update. Clin Orthop Relat Res. 2007;462:11–9.

    PubMed  Google Scholar 

  37. Yeon HB, Lindor NM, Seidman JG, Seidman CE. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome maps to chromosome 15q. Am J Hum Genet. 2000;66:1443–8.

    CAS  PubMed Central  PubMed  Google Scholar 

  38. Li J, Nishizawa K, An W, Hussey RE, Lialios FE, Salgia R, et al. A cdc15-like adaptor protein (CD2BP1) interacts with the CD2 cytoplasmic domain and regulates CD2-triggered adhesion. EMBO J. 1998;17:7320–36.

    CAS  PubMed Central  PubMed  Google Scholar 

  39. Baum W, Kirkin V, Mateus Fernandez SB, et al. Binding of the intracellular Fas ligand (FasL) domain to the adaptor protein PSTPIP results in a cytoplasmic localization of FasL. J Biol Chem. 2005;280:4012–24.

    Google Scholar 

  40. Ferguson PJ, Bing X, Vasef MA, et al. A missense mutation in pstpip2 is associated with the murine autoinflammatory disorder chronic multifocal osteomyelitis. Bone. 2006;38:41–7.

    CAS  PubMed Central  PubMed  Google Scholar 

  41. Byrd L, Grossmann M, Potter M, Shen-Ong G. Chronic multifocal osteomyelitis, a new recessive mutation on chromosome 18 of the mouse. Genomics. 1991;11:794–8.

    CAS  PubMed  Google Scholar 

  42. Colina M, Govoni M, Orzincolo C, Trotta F. Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects. Arthritis Rheum. 2009;61:813–21.

    PubMed  Google Scholar 

  43. Hayem G, Bouchaud-Chabot A, Benali K, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum. 1999;29:159–71.

    CAS  PubMed  Google Scholar 

  44. Mann B, Shaerf DA, Sheeraz A, et al. SAPHO syndrome presenting as widespread bony metastatic disease of unknown origin. Rheumatol Int. 2012;32(2):505–7.

    PubMed  Google Scholar 

  45. Sallés M, Olivé A, Perez-Andres R, et al. The SAPHO syndrome: a clinical and imaging study. Clin Rheumatol. 2011;30:245–9.

    PubMed  Google Scholar 

  46. Kahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol. 1994;8:333–62. Semin Arthritis Rheum 1999;293:159–171.

    CAS  PubMed  Google Scholar 

  47. Takigawa T, Tanaka M, Nakahara S, et al. SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically. Eur Spine J. 2008;17 Suppl 2:S331–7.

    PubMed  Google Scholar 

  48. Magrey M, Khan MA. New insights into synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Curr Rheumatol Rep. 2009;11:329–33.

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  50. Earwaker JWS, Cotten A. SAPHO: syndrome or concept? Imaging findings. Skelet Radiol. 2003;32:311–27.

    CAS  Google Scholar 

  51. Karadag-Saygi E, Gunduz OH, Gumrukcu G, et al. SAPHO syndrome: misdiagnosed and operated. Acta Reumatol Port. 2008;33:460–3.

    PubMed  Google Scholar 

  52. Court C, Charlez C, Molina V, et al. Isolated thoracic spine lesion: is this the presentation of a SAPHO syndrome? A case report. Eur Spine J. 2005;14:711–5.

    PubMed Central  PubMed  Google Scholar 

  53. Mandell GA, Contreras SJ, Conard K, Harcke HT, Maas KW. Bone scintigraphy in the detection of chronic recurrent multifocal osteomyelitis. J Nucl Med. 1998;39:1778–83.

    CAS  PubMed  Google Scholar 

  54. Khanna L, El-khoury GY. SAPHO syndrome—a pictorial assay. Iowa Orthop J. 2012;32:189–95.

    PubMed Central  PubMed  Google Scholar 

  55. Callen JP. Neutrophilic dermatoses. Dermatol Clin. 2002;20:409–19.

    PubMed  Google Scholar 

  56. Cotten A, Flipo RM, Mentre A, et al. SAPHO syndrome. Radiographics. 1995;15:1147–54.

    CAS  PubMed  Google Scholar 

  57. Sugimoto H, Tamura K, Fujii T. The SAPHO syndrome: defining the radiological spectrum of disease comprising the syndrome. Eur Radiol. 1998;8:800–6.

    CAS  PubMed  Google Scholar 

  58. Benhamou CL, Chamot AM, Kahn MF. Synovitis-acne-pustulosis-hyperostosis-osteomyelitis syndrome (SAPHO). A new syndrome among the spondyloarthropathies? Clin Exp Rheumatol. 1988;6:109–12.

    CAS  PubMed  Google Scholar 

  59. Maugars Y, Berthelot JM, Ducloux JM, et al. SAPHO syndrome: a follow-up study of 19 cases with special emphasis on enthesis involvement. J Rheumatol. 1995;22:2135–41.

    CAS  PubMed  Google Scholar 

  60. Fritz J, Tzaribatchev N, Claussen CD, et al. Chronic recurrent multifocal osteomyelitis: comparison of whole-body MR imaging with radiography and correlation with clinical and laboratory data. Radiology. 2009;252:842–51.

    PubMed  Google Scholar 

  61. Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME. The SAPHO syndrome. Semin Arthritis Rheum. 2012;42:254–65.

    PubMed  Google Scholar 

  62. Guérin-Pfyffer S, Guillaume-Czitrom S, Tammam S, Koné-Paut I. Evaluation of chronic recurrent multifocal osteitis in children by whole-body magnetic resonance imaging. Joint Bone Spine. 2012;79(6):616–20.

    PubMed  Google Scholar 

  63. Weckbach S. Whole-body MRI for inflammatory arthritis and other multifocal rheumatoid diseases. Semin Musculoskelet Radiol. 2012;16(5):377–88.

    PubMed  Google Scholar 

  64. Freyschmidt J, Sternberg A. The bullhead sign: scintigraphic pattern of sternocostoclavicular hyperostosis and pustulotic arthroosteitis. Eur Radiol. 1998;8:807–12.

    CAS  PubMed  Google Scholar 

  65. Pichler R, Weiglein K, Schmekal B, Sfetsos K, Maschek W. Bone scintigraphy using Tc-99m DPD and F18-FDG in a patient with SAPHO syndrome. Scand J Rheumatol. 2003;32:58–60.

    PubMed  Google Scholar 

  66. Inoue K, Yamaguchi T, Ozawa H, et al. Diagnosing active inflammation in the SAPHO syndrome using 18FDG-PET/CT in suspected metastatic vertebral bone tumors. Ann Nucl Med. 2007;21(8):477–80.

    PubMed  Google Scholar 

  67. Takeuchi K, Matsusita M, Takagishi K. A case of SAPHO (synovitis-acne-pustulosis-hyperostosis-osteomyelitis) syndrome in which [18F]fluorodeoxyglucose positron emission tomography was useful for differentiating from multiple metastatic bone tumors. Mod Rheumatol. 2007;17(1):67–71.

    PubMed  Google Scholar 

  68. Patel CN, Smith JT, Rankine JJ, et al. F-18 FDG PET/CT can help differentiate SAPHO syndrome from suspected metastatic bone disease. Clin Nucl Med. 2009;34:254–7.

    PubMed  Google Scholar 

  69. Canbaz F, Gonullu G, Baris S, Selcuk MB, Bicakci N. SAPHO syndrome without dermatologic manifestations: multifocal uptake mismatch on 99mTc-MDP and 18F-FDG-PET/CT imaging. Hell J Nucl Med. 2010;13(1):73–5.

    PubMed  Google Scholar 

  70. Adams MC, Turkington TG, Wilson JM, Wong TZ. A systematic review of the factors affecting accuracy of SUV measurements. AJR Am J Roentgenol. 2010;195(2):310–20.

    PubMed  Google Scholar 

  71. van Holsbeeck M, Martel W, Dequeker J, et al. Soft tissue involvement, mediastinal pseudotumor, and venous thrombosis in pustulotic arthroosteitis. A study of eight new cases. Skelet Radiol. 1989;18:1–8.

    Google Scholar 

  72. Girschick HJ, Krauspe R, Tschammler A, Huppertz HI. Chronic recurrent osteomyelitis with clavicular involvement in children: diagnostic value of different imaging techniques and therapy with non-steroidal anti-inflammatory drugs. Eur J Pediatr. 1998;157:28–33.

    CAS  PubMed  Google Scholar 

  73. Khanna G, Sato TS, Ferguson P. Imaging of chronic recurrent multifocal osteomyelitis. Radiographics. 2009;29(4):1159–77.

    PubMed  Google Scholar 

  74. Toussirot E, Dupond JL, Wendling D. Spondylodiscitis in SAPHO syndrome. A series of eight cases. Ann Rheum Dis. 1997;56:52–8.

    CAS  PubMed Central  PubMed  Google Scholar 

  75. Laredo JD, Vuillemin-Bodaghi V, Boutry N, Cotton A, Parlier-Cuau C. SAPHO syndrome: MR appearance of vertebral involvement. Radiology. 2007;242(3):825–31.

    PubMed  Google Scholar 

  76. Romanus R, Yden S. Destructive and ossifying spondylitic changes in rheumatoid ankylosing spondylitis (pelvospondylitis ossificans). Acta Orthop Scand. 1952;22:88–99.

    CAS  PubMed  Google Scholar 

  77. Hermann KG, Althoff CE, Schneider U, et al. Spinal changes in patients with spondyloarthritis: comparison of MR imaging and radiographic appearances. RadioGraphics. 2005;253:559–69.

    Google Scholar 

  78. Jevtic V, Kos-Golja M, Rozman B, McCall I. Marginal erosive discovertebral “Romanus” lesions in ankylosing spondylitis demonstrated by contrast enhanced Gd-DTPA magnetic resonance imaging. Skelet Radiol. 2000;29:27–33.

    CAS  Google Scholar 

  79. Bennet AN, Rehman A, Hensor EM, Marzo-Ortega H, Emery P, McGonagle D. The fatty Romanus lesion: a non-inflammatory spinal MRI lesion specific for axial spondyloarthropathy. Ann Rheum Dis. 2010;69(5):891–4.

    Google Scholar 

  80. Nachtigal A, Cardinal E, Bureau NJ, Sainte-Marie LG, Milette F. Vertebral involvement in SAPHO syndrome: MRI findings. Skelet Radiol. 1999;28:163–8.

    CAS  Google Scholar 

  81. Remedios D, Natali C, Saifuddin A. Case report: MRI of vertebral osteitis in early ankylosing spondylitis. Clin Radiol. 1998;53:534–6.

    CAS  PubMed  Google Scholar 

  82. Jevtic V, Rozman B, Kos-Golja M, Watt I. MR imaging in seronegative spondyloarthritis. Radiologe. 1996;36:624–31.

    CAS  PubMed  Google Scholar 

  83. Marc V, Dromer C, Le Guennec P, Manelfe C, Fournie B. Magnetic resonance imaging and axial involvement in spondylarthropathies: delineation of the spinal entheses. Rev Rhum (Engl Ed). 1997;64:465–73.

    CAS  Google Scholar 

  84. Lacout A, Rousselin B, Pelage JP. CT and MRI of spine and sacroiliac involvement in spondyloarthropathy. Am J Roentgenol. 2008;191:1016–23.

    Google Scholar 

  85. Kotilainen P, Gullichsen RE, Saario R, Manner I, Kotilainen E. Aseptic spondylitis as the initial manifestation of the SAPHO syndrome. Eur Spine J. 1997;6(5):327–9.

    CAS  PubMed Central  PubMed  Google Scholar 

  86. Sweeney SA, Kumar VA, Tayar J, et al. Case 181: synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome. Radiology. 2012;263(2):613–7.

    PubMed  Google Scholar 

  87. Nikolaisen C, Nossent H. Early histology in ankylosing spondylitis related spondylodiscitis supports its inflammatory origin. Scand J Rheumatol. 2005;34(5):396–8.

    CAS  PubMed  Google Scholar 

  88. Tohme-Noun C, Feydy A, Belmatoug N, Krainik A, Fantin B, Menu Y. Cervical involvement in SAPHO syndrome: imaging findings with a 10-year follow-up. Skelet Radiol. 2003;32:103–6.

    CAS  Google Scholar 

  89. Andersson O. Röntgenbilden vid spondylarthritis ankylopoetica. Nord Med Tidskr. 1937;14:2000–2.

    Google Scholar 

  90. Langlois S, Cedoz JP, Lohse A, Toussirot E, Wendling D. Aseptic discitis in patients with ankylosing spondylitis: a retrospective study of 14 cases. Joint Bone Spine. 2005;72(3):248–53.

    PubMed  Google Scholar 

  91. Bron JL, de Vries MK, Snieders MN, van der Horst-Bruinsma IE, van Royen BJ. Discovertebral (Andersson) lesions of the spine in ankylosing spondylitis revisited. Clin Rheumatol. 2009;28(8):883–92.

    PubMed Central  PubMed  Google Scholar 

  92. Yu L, Kasser JR, O’Rourke E, Kozakewich H. Chronic recurrent osteomyelitis. Association with vertebra plana. J Bone Joint Surg Am. 1989;71:105–12.

    CAS  PubMed  Google Scholar 

  93. Jurik AG, Egund N. MRI in chronic recurrent multifocal osteomyelitis. Skelet Radiol. 1997;26:230–8.

    CAS  Google Scholar 

  94. Demharter J, Bohndorf K, Michl W, Vogt H. Chronic recurrent multifocal osteomyelitis: a radiological and clinical investigation of five cases. Skelet Radiol. 1997;26:579–88.

    CAS  Google Scholar 

  95. Anderson SE, Heini P, Sauvain MJ. Imaging of chronic recurrent multifocalosteomyelitis of childhood first presenting with isolated primary spinal involvement. Skelet Radiol. 2003;32(6):328–36.

    CAS  Google Scholar 

  96. Huber AM, Lam PY, Duffy CM, et al. Chronic recurrent multifocal osteomyelitis: clinical outcomes after more than 5 years of follow-up. J Pediatr. 2002;141:198–203.

    PubMed  Google Scholar 

  97. Manson D, Wilmot DM, King S, Laxer RM. Physeal involvement in chronic recurrent multifocal osteomyelitis. Pediatr Radiol. 1989;20:76–9.

    CAS  PubMed  Google Scholar 

  98. Beretta-Piccoli BC, Sauvain MJ, Gal I, et al. Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in childhood: a report of ten cases and review of the literature. Eur J Pediatr. 2000;159:594–601.

    CAS  PubMed  Google Scholar 

  99. Kodama Y, Tanaka R, Kurokawa A. Severe destruction of the temporomandibular joint with complete resorption of the condyle associated with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;116(2):e128–33.

    PubMed  Google Scholar 

  100. Müller-Richter UD, Roldán JC, Mörtl M, Behr M, Reichert TE, Driemel O. SAPHO syndrome with ankylosis of the temporomandibular joint. Int J Oral Maxillofac Surg. 2009;38(12):1335–41.

    PubMed  Google Scholar 

  101. Björkstén B, Boquist L. Histopathological aspects of chronic recurrent multifocal osteomyelitis. J Bone Joint Surg (Br). 1980;62:376–80.

    Google Scholar 

  102. Reith JD, Bauer TW, Schils JP. Osseous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. Am J Surg Pathol. 1996;20:1368–77.

    CAS  PubMed  Google Scholar 

  103. Chow LT, Griffith JF, Kumta SM, Leung PC. Chronic recurrent multifocal osteomyelitis: a great clinical and radiologic mimic in need of recognition by the pathologist. APMIS. 1999;107:369–79.

    CAS  PubMed  Google Scholar 

  104. Rozin AP, Nahir AM. Is SAPHO syndrome a target for antibiotic therapy? Clin Rheumatol. 2007;26:817–20.

    CAS  PubMed  Google Scholar 

  105. Ballara SC, Siraj QH, Maini RN, Venables PJ. Sustained response to doxycycline therapy in two patients with SAPHO syndrome. Arthritis Rheum. 1999;42:819–21.

    CAS  PubMed  Google Scholar 

  106. Schilling F, Wagner AD. Azithromycin: an anti-inflammatory effect in chronic recurrent multifocal osteomyelitis? A preliminary report. Z Rheumatol. 2000;59:352–3.

    CAS  PubMed  Google Scholar 

  107. Just A, Adams S, Brinkmeier T, et al. Successful treatment of primary chronic osteomyelitis in SAPHO syndrome with bisphosphonates. Dtsch Dermatol Ges. 2008;6(8):657–60.

    Google Scholar 

  108. Amital H, Applbaum YH, Aamar S, Daniel N, Rubinow A. SAPHO syndrome treated with pamidronate: an open-label study of 10 patients. Rheumatology (Oxford). 2004;43(5):658–61.

    CAS  Google Scholar 

  109. Solau-Gervais E, Soubrier M, Gerot I, et al. The usefulness of bone remodelling markers in predicting the efficacy of pamidronate treatment in SAPHO syndrome. Rheumatology (Oxford). 2006;45:339–42.

    CAS  Google Scholar 

  110. Whyte MP, Wenkert D, Clements KL, McAlister WH, Mumm S. Bisphosphonate-induced osteopetrosis. N Engl J Med. 2003;349:457–63.

    CAS  PubMed  Google Scholar 

  111. Chang JT, Green L, Beitz J. Renal failure with the use of zoledronic acid. N Engl J Med. 2003;349:1676–9.

    CAS  PubMed  Google Scholar 

  112. Rogers MJ, Gordon S, Benford HL, et al. Cellular and molecular mechanisms of action of bisphosphonates. Cancer. 2000;88(12 Suppl):2961–78.

    CAS  PubMed  Google Scholar 

  113. Arias-Santiago S, Sanchez-Cano D, Callejas-Rubio JL, et al. Adalimumab treatment for SAPHO syndrome. Acta Derm Venereol. 2010;90:301–2.

    PubMed  Google Scholar 

  114. Deutschmann A, Mache CJ, Bodo K, et al. Successful treatment of chronic recurrent multifocal osteomyelitis with tumor necrosis factor-alpha blockage. Pediatrics. 2005;116:1231–3.

    PubMed  Google Scholar 

  115. Ben Abdelghani K, Dran DG, Gottenberg JE, et al. Tumor necrosis factor alpha blockers in SAPHO syndrome. J Rheumatol. 2010;37:1699–704.

    CAS  PubMed  Google Scholar 

  116. Moll C, Hernández MV, Cañete JD, et al. Ilium Osteitis as the main manifestation of the SAPHO syndrome: response to infliximab therapy and review of the literature. Semin Arthritis Rheum. 2008;37(5):299–306.

    CAS  PubMed  Google Scholar 

  117. Braun J, Baraliakos X, Brandt J, Listing J, Zink A, Alten R, et al. Persistent clinical response to the anti-TNF-alpha antibody infliximab in patients with ankylosing spondylitis over 3 years. Rheumatology. 2005;44:670–6.

    CAS  PubMed  Google Scholar 

  118. Wollina U, Hansel G, Koch A, Schönlebe J, Köstler E, Haroske G. Tumor necrosis factor-alpha inhibitor-induced psoriasis or psoriasiform exanthemata: first 120 cases from the literature including a series of six new patients. Am J Clin Dermatol. 2008;9:1–14.

    PubMed  Google Scholar 

  119. Aksentijevich I, Masters SL, Ferguson PJ, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med. 2009;360:2426–37.

    CAS  PubMed Central  PubMed  Google Scholar 

  120. Reddy S, Jia S, Geoffrey R, et al. An autoinflammatory disease due to homozygous deletion of the IL1RN locus. N Engl J Med. 2009;360:2438–44.

    CAS  PubMed Central  PubMed  Google Scholar 

  121. Burgermeister LT, Baeten DL, Tas SW. Biologics for rare inflammatory diseases: TNF blockade in the SAPHO syndrome. Neth J Med. 2012;70(10):444–9.

    Google Scholar 

  122. Ferguson PJ, Sandu M. Current understanding of the pathogenesis and management of chronic recurrent multifocal osteomyelitis. Curr Rheumatol Rep. 2012;14(2):130–41.

    CAS  PubMed Central  PubMed  Google Scholar 

  123. Rech J, Manger B, Lang B, Schett G, Wilhelm M, Birkmann J. Adult-onset Still’s disease and chronic recurrent multifocal osteomyelitis: a hitherto undescribed manifestation of autoinflammation. Rheumatol Int. 2012;32(6):1827–9.

    CAS  PubMed  Google Scholar 

  124. Colina M, Pizzirani C, Khodeir M, et al. Dysregulation of P2X7 receptor-inflammasome axis in SAPHO syndrome: successful treatment with anakinra. Rheumatology. 2010;49:1416–8.

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Radiological Sciences, Catholic University, School of Medicine, Largo A. Gemelli 1, 00168, Rome, Italy

    Antonio Leone, Roberto Casale, Nicola Magarelli, Alessia Semprini & Cesare Colosimo

  2. Department of Diagnostic Imaging, The Robert Jones and Agnes Hunt Orthopaedic and District Hospital, Oswestry, Shropshire, England, UK

    Victor N. Cassar-Pullicino

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  1. Antonio Leone
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  2. Victor N. Cassar-Pullicino
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  3. Roberto Casale
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  6. Cesare Colosimo
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Correspondence to Antonio Leone.

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Leone, A., Cassar-Pullicino, V.N., Casale, R. et al. The SAPHO syndrome revisited with an emphasis on spinal manifestations. Skeletal Radiol 44, 9–24 (2015). https://doi.org/10.1007/s00256-014-2025-0

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  • DOI: https://doi.org/10.1007/s00256-014-2025-0

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