Book chapter
46 - Autoinflammatory Bone Diseases
Textbook of Pediatric Rheumatology, pp.643-659
Elsevier, Ninth Edition
2026
DOI: 10.1016/B978-0-443-11980-4.00046-5
Abstract
Autoinflammatory disorders (covered extensively in Chapter 45) result from aberrant and/or spontaneous activation of components of the innate immune system. At least initially, they occur in the absence of high-titer autoantibodies or autoreactive lymphocytes. This, by definition, distinguishes them from classic autoimmune disorders. Autoantibodies and autoreactive lymphocytes, however, may develop over the course of disease partially as a result of tissue damage and exposure of the immune system to intracellular components. The concept of autoinflammatory disorders was proposed in 1999, after the identification of the genetic basis of the prototypic periodic fever syndromes, familial Mediterranean fever, and tumor necrosis factor receptor–associated periodic syndrome. There are now more than 50 disorders that are thought to be autoinflammatory, most of which present in childhood; included are a group of disorders that have bone inflammation as a main phenotypic feature including chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis; synovitis, acne, pustulosis, hyperostosis, osteitis syndrome; Majeed syndrome; deficiency of interleukin-1 receptor antagonist, and cherubism.
Details
- Title: Subtitle
- 46 - Autoinflammatory Bone Diseases
- Creators
- Polly J. FergusonChristian M. Hedrich
- Resource Type
- Book chapter
- Publication Details
- Textbook of Pediatric Rheumatology, pp.643-659
- Edition
- Ninth Edition
- DOI
- 10.1016/B978-0-443-11980-4.00046-5
- Publisher
- Elsevier; Amsterdam
- Language
- English
- Date published
- 2026
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9985166967302771
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