title: Chronic Recurrent Multifocal Osteomyelitis and Related Diseases-Update on Pathogenesis.
abstract: OBJECTIVE
  We focus on recent advances in the understanding of the genetic, molecular, immunologic, and environmental factors implicated in the pathogenesis of autoinflammatory bone diseases including the syndromic and non-syndromic forms of chronic recurrent multifocal osteomyelitis (CRMO).
  
  
  RESULTS
  Evidence implicating the IL-1 pathway in the pathogenesis of the Mendelian forms of CRMO is growing. LIPIN2 can regulate the NLRP3 inflammasome by affecting P2X7 receptor activation, and intracellular cholesterol can modulate P2X7R currents. Work in a mouse model of CRMO demonstrates that dietary manipulation can alter the microbiome and protect these mice from the development of sterile osteomyelitis in vivo. Although the genetic and immunologic basis of non-syndromic CRMO remains only partially understood, the IL-1 pathway is central to the pathogenesis in the syndromic autoinflammatory bone disorders. Recent work implicates lipids and the microbiome in sterile osteomyelitis.
authors.affiliation: Department of Pediatrics and the Inflammation Program, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
authors.forename: Allison J
authors.initials: AJ
authors.lastname: Cox
authors.affiliation: Pediatric Rheumatology, Department of Pediatrics, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
authors.forename: Yongdong
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Pediatrics and the Inflammation Program, University of Iowa Carver College of Medicine, Iowa City, IA, USA. polly-ferguson@uiowa.edu.
authors.forename: Polly J
authors.initials: PJ
authors.lastname: Ferguson
mesh_terms: D000742:Anemia, Dyserythropoietic, Congenital / Q000209:etiology; D000818:Animals; D015815:Cell Adhesion Molecules / Q000235:genetics; D003598:Cytoskeletal Proteins / Q000235:genetics; D004195:Disease Models, Animal; D056660:Hereditary Autoinflammatory Diseases / Q000209:etiology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes; D058847:Inflammasomes / Q000502:physiology; D007375:Interleukin-1 / Q000276:immunology; D051379:Mice; D064307:Microbiota; D009687:Nuclear Proteins / Q000502:physiology; D010019:Osteomyelitis / Q000209:etiology*
chemical_list: D015815:Cell Adhesion Molecules; D003598:Cytoskeletal Proteins; C473794:FBLIM1 protein, human; D058847:Inflammasomes; D007375:Interleukin-1; C502575:LPIN2 protein, human; D009687:Nuclear Proteins
keywords: Autoinflammatory; Chronic non-bacterial osteomyelitis; Chronic recurrent osteomyelitis; Inflammasome; Interleukin-1