title: Autoinflammatory bone diseases.
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.
authors.affiliation: Division of Immunology, Rheumatology, and Allergy, Department of Pediatrics, University of Utah, PO Box 581289, Salt Lake City, UT 84158, USA. Electronic address: Sara.Stern@hsc.utah.edu.
authors.forename: Sara M
authors.initials: SM
authors.lastname: Stern
authors.forename: Polly J
authors.initials: PJ
authors.lastname: Ferguson
mesh_terms: D000152:Acne Vulgaris / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D020083:Acquired Hyperostosis Syndrome / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D000742:Anemia, Dyserythropoietic, Congenital / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D001847:Bone Diseases / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D002636:Cherubism / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D002908:Chronic Disease; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D006801:Humans; D015576:Hyperostosis / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D007153:Immunologic Deficiency Syndromes; D007249:Inflammation; D053590:Interleukin 1 Receptor Antagonist Protein / Q000276:immunology; D010000:Osteitis / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D010019:Osteomyelitis / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D013577:Syndrome; D013585:Synovitis / Q000175:diagnosis / Q000276:immunology / Q000628:therapy
chemical_list: D053590:Interleukin 1 Receptor Antagonist Protein
keywords: Autoinflammatory bone diseases; CRMO; Cherubism; Chronic nonbacterial osteomyelitis; DIRA; Majeed syndrome; SAPHO syndrome