Skip to main content
SpringerLink
Log in

SAPHO Syndrome

  • Chapter
  • First Online:
Rarer Arthropathies

Part of the book series: Rare Diseases of the Immune System ((RDIS))

  • 387 Accesses

Abstract

Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome is a rare and complex multisystem autoimmune condition with a unique clinical presentation. It typically starts in the young to middle aged, causing pustular dermatosis and inflammation of the bone, joints and entheses of the anterior chest wall. SAPHO involves a unique pattern of innate immune system dysregulation and likely microbial interaction, sharing some pathological mechanisms with spondyloarthritis and pustular dermatosis. Diagnosis requires recognition of characteristic features and the exclusion of mimics including infection, malignancy and other autoimmune diseases. Characteristic bone changes may be seen on radiography, CT, MRI or bone scan. Acute treatment commonly involves anti-inflammatory agents, corticosteroids, or bisphosphonates while prolonged disease activity may warrant biologic medications. Tumour necrosis factor inhibitors have the most supporting data from case series, and other biologics used to treat inflammatory arthritis and pustular skin disease have encouraging case reports.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. 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(3):187–96.

    Google Scholar 

  2. Przepiera-Będzak H, Brzosko M. Clinical symptoms, imaging, and treatment of SAPHO syndrome: a single-center study of 52 cases. Pol Arch Intern Med. 2018;128(6):396–9.

    PubMed  Google Scholar 

  3. Takigawa T, Tanaka M, Nakanishi K, Misawa H, Sugimoto Y, Takahata T, et al. SAPHO syndrome associated spondylitis. Eur Spine J. 2008;17(10):1391–7.

    Article  Google Scholar 

  4. Yu M, Cao Y, Li J, Zhang Y, Ye Y, Wang L, et al. Anterior chest wall in SAPHO syndrome: magnetic resonance imaging findings. Arthritis Res Ther. 2020;22(1):216.

    Article  CAS  Google Scholar 

  5. 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(6):813–21.

    Article  Google Scholar 

  6. Furer V, Kishimoto M, Tsuji S, Taniguchi Y, Ishihara Y, Tomita T, et al. The diagnosis and treatment of adult patients with SAPHO syndrome: controversies revealed in a multidisciplinary international survey of physicians. Rheumatol Ther. 2020;7(4):883–91.

    Article  Google Scholar 

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

    Article  Google Scholar 

  8. Xu D, Liu X, Lu C, Luo J, Wang C, Gao C, et al. Reduction of peripheral natural killer cells in patients with SAPHO syndrome. Clin Exp Rheumatol. 2019;37(1):12–8.

    PubMed  Google Scholar 

  9. Zhang S, Li C, Zhang S, Li L, Zhang W, Dong Z, et al. Serum levels of proinflammatory, anti-inflammatory cytokines, and RANKL/OPG in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Mod Rheumatol. 2019;29(3):523–30.

    Article  CAS  Google Scholar 

  10. Liao H-J, Chyuan I-T, Wu C-S, Lin S-W, Chen K-H, Tsai H-F, et al. Increased neutrophil infiltration, IL-1 production and a SAPHO syndrome-like phenotype in PSTPIP2-deficient mice. Rheumatol Oxf Engl. 2015;54(7):1317–26.

    Article  CAS  Google Scholar 

  11. Sahdo B, Särndahl E, Elgh F, Söderquist B. Propionibacterium acnes activates caspase-1 in human neutrophils. APMIS Acta Pathol Microbiol Immunol Scand. 2013;121(7):652–63.

    Article  CAS  Google Scholar 

  12. 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(5):R140.

    Article  Google Scholar 

  13. Li C, Ye Y, Cao Y, Zhang W, Xu W, Wu N, et al. Axial skeletal lesions and disease duration in SAPHO syndrome: a retrospective review of computed tomography findings in 81 patients. Int J Rheum Dis. 2020;23(9):1152–8.

    Article  CAS  Google Scholar 

  14. Li C, Zuo Y, Wu N, Li L, Li F, Zhang W, et al. Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre study of a cohort of 164 patients. Rheumatol Oxf Engl. 2016;55(6):1023–30.

    Article  CAS  Google Scholar 

  15. Earwaker JWS, Cotten A. SAPHO: syndrome or concept? Imaging findings. Skeletal Radiol. 2003;32(6):311–27.

    Article  CAS  Google Scholar 

  16. Gao S, Deng X, Zhang L, Song L. The comparison analysis of clinical and radiological features in SAPHO syndrome. Clin Rheumatol. 2021;40(1):349–57.

    Article  Google Scholar 

  17. 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(3):159–71.

    Article  CAS  Google Scholar 

  18. Li C, Cao Y, Zhang W. Clinical heterogeneity of SAPHO syndrome: challenge of diagnosis. Mod Rheumatol. 2018;28(3):432–4.

    Article  Google Scholar 

  19. Benhamou CL, Chamot AM, Kahn MF. Synovitis-acne-pustulosis hyperostosis-osteomyelitis syndrome (SAPHO). A new syndrome among the spondyloarthropathies? Clin Exp Rheumatol. 1988;6(2):109–12.

    CAS  PubMed  Google Scholar 

  20. Kahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol. 1994;8(2):333–62.

    Article  CAS  Google Scholar 

  21. Kahn MF. Proposed classification criteria of SAPHO syndrome. In: Proceedings of the annual meeting of the American College of Radiology, Orlando, Florida. October 23–28, 2003.

    Google Scholar 

  22. Hedrich CM, Morbach H, Reiser C, Girschick HJ. New insights into adult and paediatric chronic non-bacterial osteomyelitis CNO. Curr Rheumatol Rep. 2020;22(9):52.

    Article  Google Scholar 

  23. Colina M. Comment on “SAPHO syndrome: imaging findings of vertebral involvement”. AJNR Am J Neuroradiol. 2016;37(10):E65–6.

    Article  CAS  Google Scholar 

  24. Ross JJ, Shamsuddin H. Sternoclavicular septic arthritis: review of 180 cases. Medicine (Baltimore). 2004;83(3):139–48.

    Article  Google Scholar 

  25. Edwin J, Ahmed S, Verma S, Tytherleigh-Strong G, Karuppaiah K, Sinha J. Swellings of the sternoclavicular joint: review of traumatic and non-traumatic pathologies. EFORT Open Rev. 2018;3(8):471–84.

    Article  Google Scholar 

  26. Xu W, Li C, Zhao X, Lu J, Li L, Wu N, et al. Whole-spine computed tomography findings in SAPHO syndrome. J Rheumatol. 2017;44(5):648–54.

    Article  Google Scholar 

  27. Skrabl-Baumgartner A, Singer P, Greimel T, Gorkiewicz G, Hermann J. Chronic non-bacterial osteomyelitis: a comparative study between children and adults. Pediatr Rheumatol Online J. 2019;17(1):49.

    Article  Google Scholar 

  28. Cao Y, Li C, Yang Q, Wu N, Xu P, Li Y, et al. Three patterns of osteoarticular involvement in SAPHO syndrome: a cluster analysis based on whole body bone scintigraphy of 157 patients. Rheumatol Oxf Engl. 2019;58(6):1047–55.

    Article  Google Scholar 

  29. Sun X, Li C, Cao Y, Shi X, Li L, Zhang W, et al. F-18 FDG PET/CT in 26 patients with SAPHO syndrome: a new vision of clinical and bone scintigraphy correlation. J Orthop Surg. 2018;13(1):120.

    Article  Google Scholar 

  30. Huang H, Zhang Z, Zhao J, Hao Y, Zhou W. The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature. Clin Rheumatol. 2021;40(3):1131–9.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Coast Joint Care, Maroochydore, QLD, Australia

    Steven Truong & Peter Nash

  2. Griffith University School of Medicine and Dentistry, Brisbane, QLD, Australia

    Steven Truong & Peter Nash

Authors
  1. Steven Truong
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Peter Nash
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Steven Truong .

Editor information

Editors and Affiliations

  1. Centre for Rheumatology, Calicut, Kerala, India

    Vinod Ravindran

  2. Department of Rheumatology, Kauvery Hospitals, Chennai, Tamil Nadu, India

    Sham Santhanam

  3. CARE Pain and Arthritis Centre, Goyal Hospital, Udaipur, Rajasthan, India

    Mohit Goyal

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Truong, S., Nash, P. (2022). SAPHO Syndrome. In: Ravindran, V., Santhanam, S., Goyal, M. (eds) Rarer Arthropathies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-031-05002-2_5

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-05002-2_5

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-05001-5

  • Online ISBN: 978-3-031-05002-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation