Autoinflammatory bone diseases

Rheum Dis Clin North Am. 2013 Nov;39(4):735-49. doi: 10.1016/j.rdc.2013.05.002. Epub 2013 Aug 17.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Acne Vulgaris / diagnosis
  • Acne Vulgaris / immunology
  • Acne Vulgaris / therapy
  • Acquired Hyperostosis Syndrome / diagnosis
  • Acquired Hyperostosis Syndrome / immunology
  • Acquired Hyperostosis Syndrome / therapy
  • Anemia, Dyserythropoietic, Congenital / diagnosis
  • Anemia, Dyserythropoietic, Congenital / immunology
  • Anemia, Dyserythropoietic, Congenital / therapy
  • Bone Diseases / diagnosis
  • Bone Diseases / immunology*
  • Bone Diseases / therapy
  • Cherubism / diagnosis
  • Cherubism / immunology
  • Cherubism / therapy
  • Chronic Disease
  • Hereditary Autoinflammatory Diseases / diagnosis
  • Hereditary Autoinflammatory Diseases / immunology*
  • Hereditary Autoinflammatory Diseases / therapy
  • Humans
  • Hyperostosis / diagnosis
  • Hyperostosis / immunology
  • Hyperostosis / therapy
  • Immunologic Deficiency Syndromes
  • Inflammation
  • Interleukin 1 Receptor Antagonist Protein / immunology
  • Osteitis / diagnosis
  • Osteitis / immunology
  • Osteitis / therapy
  • Osteomyelitis / diagnosis
  • Osteomyelitis / immunology
  • Osteomyelitis / therapy
  • Syndrome
  • Synovitis / diagnosis
  • Synovitis / immunology
  • Synovitis / therapy

Substances

  • Interleukin 1 Receptor Antagonist Protein

Supplementary concepts

  • Deficiency of interleukin-1 receptor antagonist
  • Majeed syndrome