Imaging of Chronic Recurrent Multifocal Osteomyelitis and Autoinflammatory Bone Disorders

  • Sergio Lopes Viana
  • Maria Custódia Machado Ribeiro
  • Bruno Beber Machado


Autoinflammatory bone diseases are disorders characterized by chronic osteitis, bone resorption, and abnormal remodeling resulting from aberrant unprovoked activation of the innate immune system. The areas affected by chronic bone inflammation are typically culture-negative, with no apparent pathogen on histopathology. Inflammatory skin lesions are often present, as well as accompanying fever. Chronic recurrent multifocal osteomyelitis (CRMO), SAPHO syndrome, Majeed syndrome, pyoderma gangrenosum and acne (PAPA), deficiency of interleukin-1 receptor antagonist (DIRA), CINCA/NOMID, and cherubism are among the most common autoinflammatory bone disorders in childhood, and CRMO is by far the most studied and well-recognized of them all. This chapter covers the imaging findings of some of these diseases, focusing on CRMO as the prototypical condition of the group.


Imaging Joints Radiology Children Adolescents Pediatrics Radiographs X-rays Ultrasonography Computed tomography Magnetic resonance imaging Bone scintigraphy Autoinflammatory bone diseases Autoinflammatory disorders Chronic recurrent multifocal osteomyelitis CRMO SAPHO Majeed syndrome Deficiency of interleukin-1 receptor antagonist (DIRA) Chronic infantile neurological cutaneous and articular syndrome CINCA Neonatal-onset multisystem inflammatory disease NOMID Osteitis Arthritis Synovitis 

Recommended Reading

  1. 1.
    Aksentijevich I, Masters SL, Ferguson PJ, Dancey P, Frenkel J, van Royen-Kerkhoff A, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med. 2009;360:2426–37.CrossRefGoogle Scholar
  2. 2.
    Berkowitz YJ, Greenwood SJ, Cribb G, Davies K, Cassar-Pullicino VN. Complete resolution and remodeling of chronic recurrent multifocal osteomyelitis on MRI and radiographs. Skeletal Radiol. 2018;47:563–8.CrossRefGoogle Scholar
  3. 3.
    Chen HC, Wuerdeman MF, Chang JH, Nieves-Robbins NM. The role of whole-body magnetic resonance imaging in diagnosing chronic recurrent multifocal osteomyelitis. Radiol Case Rep. 2018;13:485–9.CrossRefGoogle Scholar
  4. 4.
    Costa-Reis P, Sullivan KE. Chronic recurrent multifocal osteomyelitis. J Clin Immunol. 2013;33:1043–56.CrossRefGoogle Scholar
  5. 5.
    Falip C, Alison M, Boutry N, Job-Deslandre C, Cotten A, Azoulay R, et al. Chronic recurrent multifocal osteomyelitis (CRMO): a longitudinal case series review. Pediatr Radiol. 2013;43:355–75.CrossRefGoogle Scholar
  6. 6.
    Greenwood S, Leone A, Cassar-Pullicino VN. SAPHO and recurrent multifocal osteomyelitis. Radiol Clin North Am. 2017;55:1035–53.CrossRefGoogle Scholar
  7. 7.
    Greer MC. Whole-body magnetic resonance imaging: techniques and non-oncologic indications. Pediatr Radiol. 2018;48:1348–63.CrossRefGoogle Scholar
  8. 8.
    Hedrich CM, Hofmann SR, Pablik J, Morbach H, Girschick HJ. Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO). Pediatr Rheumatol Online J. 2013;
  9. 9.
    Hill SC, Namde M, Dwyer A, Poznanski A, Canna S, Goldbach-Mansky R. Arthropathy of neonatal onset multisystem inflammatory disease (NOMID/CINCA). Pediatr Radiol. 2007;37:145–52.CrossRefGoogle Scholar
  10. 10.
    Jurik AG, Klicman RF, Simoni P, Robinson P, Teh J. SAPHO and CRMO: the value of imaging. Semin Musculoskelet Radiol. 2018;22:207–24.CrossRefGoogle Scholar
  11. 11.
    Khanna G, Sato TS, Ferguson P. Imaging of chronic recurrent multifocal osteomyelitis. Radiographics. 2009;29:1159–77.CrossRefGoogle Scholar
  12. 12.
    Koplas MC, Simonelli PS, Bauer TW, Joyce MJ, Sundaram M. Your diagnosis? chronic recurrent multifocal osteomyelitis. Orthopedics. 2010;33:218.CrossRefGoogle Scholar
  13. 13.
    Leclair N, Thörmer G, Sorge I, Ritter L, Schuster V, Hirsch FW. Whole-body diffusion-weighted imaging in chronic recurrent multifocal osteomyelitis in children. PLoS One. 2016;
  14. 14.
    Mallick A, Chaturvedi A, Muthukumar N. Chronic recurrent multifocal osteomyelitis: a diagnostic dilemma: a case report and review of the literature. JBJS Case Connect. 2016;
  15. 15.
    Modesto C. Síndrome CINCA/NOMID. Med Clin (Barc). 2011;136(Suppl 1):10–5.CrossRefGoogle Scholar
  16. 16.
    Morbach H, Hedrich CM, Beer M, Girschick HJ. Autoinflammatory bone disorders. Clin Immunol. 2013;147:185–96.CrossRefGoogle Scholar
  17. 17.
    Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME. The SAPHO syndrome. Semin Arthritis Rheum. 2012;42:254–65.CrossRefGoogle Scholar
  18. 18.
    Queiroz RM, Rocha PHP, Lauar LZ, Costa MJBD, Laguna CB, Oliveira RGG. Chronic recurrent multifocal osteomyelitis exhibiting predominance of periosteal reaction. Rev Assoc Med Bras. 1992;63:303–6.CrossRefGoogle Scholar
  19. 19.
    Roderick MR, Shah R, Rogers V, Finn A, Ramanan AV. Chronic recurrent multifocal osteomyelitis (CRMO) – advancing the diagnosis. Pediatr Rheumatol Online J. 2016;
  20. 20.
    Roderick MR, Sen ES, Ramanan AV. Chronic recurrent multifocal osteomyelitis in children and adults: current understanding and areas for development. Rheumatology (Oxford). 2018;57:41–8.CrossRefGoogle Scholar
  21. 21.
    Rukavina I. SAPHO syndrome: a review. J Child Orthop. 2015;9:19–27.CrossRefGoogle Scholar
  22. 22.
    Sirisha K, Keerthi T, Habibi S, Varaprasad IR, Jyotsna Rani Y, Narendranath L, et al. Chronic recurrent multifocal osteomyelitis complicated by hip subluxation: a case report. Int J Rheum Dis. 2017;20:1800–1.CrossRefGoogle Scholar
  23. 23.
    Stern SM, Ferguson PJ. Autoinflammatory bone diseases. Rheum Dis Clin North Am. 2013;39:735–49.CrossRefGoogle Scholar
  24. 24.
    Surendra G, Shetty U. Chronic recurrent multifocal osteomyelitis: a rare entity. J Med Imaging Radiat Oncol. 2015;59:436–44.CrossRefGoogle Scholar
  25. 25.
    Thacker PG, Binkovitz LA, Thomas KB. Deficiency of interleukin-1-receptor antagonist syndrome: a rare auto-inflammatory condition that mimics multiple classic radiographic findings. Pediatr Radiol. 2012;42:495–8.CrossRefGoogle Scholar
  26. 26.
    Vargas Pérez M, Sevilla Pérez B. SAPHO syndrome in childhood. A case report. Reumatol Clin 2018;14:109–12.Google Scholar
  27. 27.
    Voit AM, Arnoldi AP, Douis H, Bleisteiner F, Jansson MK, Reiser MF, et al. Whole-body magnetic resonance imaging in chronic recurrent multifocal osteomyelitis: clinical longterm assessment may underestimate activity. J Rheumatol. 2015;42:1455–62.CrossRefGoogle Scholar
  28. 28.
    von Kalle T, Heim N, Hospach T, Langendörfer M, Winkler P, Stuber T. Typical patterns of bone involvement in whole-body MRI of patients with chronic recurrent multifocal osteomyelitis (CRMO). Rofo. 2013;185:655–61.CrossRefGoogle Scholar
  29. 29.
    Zaki FM, Sridharan R, Pei TS, Ibrahim S, Ping TS. NOMID: the radiographic and MRI features and review of literature. J Radiol Case Rep. 2012;6:1–8.PubMedPubMedCentralGoogle Scholar
  30. 30.
    Zhao Y, Ferguson PJ. Chronic nonbacterial osteomyelitis and chronic recurrent multifocal osteomyelitis in children. Pediatr Clin North Am. 2018;65:783–800.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Sergio Lopes Viana
    • 1
  • Maria Custódia Machado Ribeiro
    • 2
  • Bruno Beber Machado
    • 3
  1. 1.Hospital da Criança de Brasília José Alencar, Hospital Ortopédico e Medicina Especializada (HOME) and Diagnóstico Clínica de Imagens MédicasBrasíliaBrazil
  2. 2.Hospital da Criança de Brasília José AlencarBrasíliaBrazil
  3. 3.Gastren Clínica Médica e Radiológica (Iúna-ES), Clínica Radiológica Med Imagem, Clínica Radiológica Radial, Santa Casa de Misericórdia and Unimed Sul CapixabaCachoeiro de ItapemirimBrazil

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