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Chronic Recurrent Multifocal Osteomyelitis and Related Diseases-Update on Pathogenesis.

ABSTRACT: 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).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.

SUBMITTER: Cox AJ

PROVIDER: S-EPMC5545929 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature

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Chronic Recurrent Multifocal Osteomyelitis and Related Diseases-Update on Pathogenesis.

Cox Allison J AJ Zhao Yongdong Y Ferguson Polly J PJ

Current rheumatology reports 20170401 4

<h4>Purpose of review</h4>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).<h4>Recent findings</h4>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 recepto ...[more]

PMID: 28361334

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Chronic Recurrent Multifocal Osteomyelitis (CRMO): Presentation, Pathogenesis, and Treatment.

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Analysis of the fecal and oral microbiota in chronic recurrent multifocal osteomyelitis

Project description:Analysis of the fecal and oral microbiota in chronic recurrent multifocal osteomyelitis

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Genetic variants in FBLIM1 gene do not contribute to SAPHO syndrome and chronic recurrent multifocal osteomyelitis in typical patient groups.

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Homozygous mutations in LPIN2 are responsible for the syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia (Majeed syndrome).

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