Journal article
Autoinflammatory bone disorders
Current opinion in rheumatology, Vol.19(5), pp.492-498
09/2007
DOI: 10.1097/BOR.0b013e32825f5492
PMID: 17762617
Abstract
PURPOSE OF REVIEWThis review provides an update on clinical, genetic, and immunologic aspects of the autoinflammatory bone disorders.
RECENT FINDINGSChronic 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.
SUMMARYChronic 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.
Details
- Title: Subtitle
- Autoinflammatory bone disorders
- Creators
- Polly Ferguson - Department of Pediatrics, University of Iowa, Iowa City, Iowa, USAHatem El-Shanti
- Resource Type
- Journal article
- Publication Details
- Current opinion in rheumatology, Vol.19(5), pp.492-498
- Publisher
- Lippincott Williams & Wilkins, Inc
- DOI
- 10.1097/BOR.0b013e32825f5492
- PMID
- 17762617
- ISSN
- 1040-8711
- eISSN
- 1531-6963
- Language
- English
- Date published
- 09/2007
- Academic Unit
- Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Medical Genetics and Genomics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984065739602771
Metrics
18 Record Views