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Review
. 2013 Nov;39(4):735-49.
doi: 10.1016/j.rdc.2013.05.002. Epub 2013 Aug 17.

Autoinflammatory bone diseases

Sara M Stern  1 , Polly J Ferguson
Affiliations
Review

Autoinflammatory bone diseases

Sara M Stern et al. Rheum Dis Clin North Am. 2013 Nov.

Abstract

Autoinflammatory bone disease is a new branch of autoinflammatory diseases caused by seemingly unprovoked activation of the innate immune system leading to an osseous inflammatory process. The inflammatory bone lesions in these disorders are characterized by chronic inflammation that is typically culture negative with no demonstrable organism on histopathology. The most common autoinflammatory bone diseases in childhood include chronic nonbacterial osteomyelitis (CNO), synovitis, acne, pustulosis, hyperostosis, osteitis syndrome, Majeed syndrome, deficiency of interleukin-1 receptor antagonist, and cherubism. In this article, the authors focus on CNO and summarize the distinct genetic autoinflammatory bone syndromes.

Keywords: Autoinflammatory bone diseases; CRMO; Cherubism; Chronic nonbacterial osteomyelitis; DIRA; Majeed syndrome; SAPHO syndrome.

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Figures

Figure 1
Figure 1
Figure 2
Figure 2
Majeed's Clinical Triad
Figure 3
Figure 3
Summary of Clinical Course in DIRA
See this image and copyright information in PMC

References

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    1. Borzutzky A, Stern S, Reiff A, et al. Pediatric chronic nonbacterial osteomyelitis. Pediatrics. 2012;130:e1190–e1197. - PubMed

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