Autoinflammatory bone disorders

Polly J. Ferguson, Hatem I. El-Shanti

Research output: Contribution to journalArticle

96 Citations (Scopus)

Abstract

PURPOSE OF REVIEW: This review provides an update on clinical, genetic, and immunologic aspects of the autoinflammatory bone disorders. RECENT FINDINGS: Chronic noninfectious inflammation of the bone is a clinical feature of both chronic recurrent multifocal osteomyelitis and (to a lesser degree) cherubism. The genes responsible for Majeed syndrome (LPIN2), murine chronic multifocal osteomyelitis (pstpip2), and cherubism (SH3BP2 and possibly PTPN11) have been identified. Murine models of both chronic recurrent multifocal osteomyelitis and cherubism have demonstrated that the bone inflammation is mediated by hematopoietically derived cells and can occur in the absence of a functioning adaptive immune system. As the immunologic defects become better defined, the cells of the myeloid lineage are emerging as the primary players. SUMMARY: Chronic multifocal osteomyelitis and cherubism are hereditary chronic inflammatory disorders in which bone is the primary inflammatory target. Recent genetic and immunologic discoveries demonstrate involvement of the innate immune system, which places these entities in the category of autoinflammatory disorders.

Original languageEnglish
Pages (from-to)492-498
Number of pages7
JournalCurrent Opinion in Rheumatology
Volume19
Issue number5
DOIs
Publication statusPublished - Sep 2007
Externally publishedYes

Fingerprint

Cherubism
Bone and Bones
Osteitis
Immune System
Cell Lineage
Chronic recurrent multifocal osteomyelitis
Genes

Keywords

  • Autoinflammatory
  • Cherubism
  • Chronic recurrent multifocal osteomyelitis
  • Genetics
  • Osteomyelitis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Autoinflammatory bone disorders. / Ferguson, Polly J.; El-Shanti, Hatem I.

In: Current Opinion in Rheumatology, Vol. 19, No. 5, 09.2007, p. 492-498.

Research output: Contribution to journalArticle

Ferguson, Polly J. ; El-Shanti, Hatem I. / Autoinflammatory bone disorders. In: Current Opinion in Rheumatology. 2007 ; Vol. 19, No. 5. pp. 492-498.
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