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Chronic Recurrent Multifocal Osteomyelitis and Related Diseases-Update on Pathogenesis
Allison J Cox1, Yongdong Zhao2, Polly J Ferguson3
1Department of Pediatrics and the Inflammation Program, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Current Rheumatology Reports
|April 1, 2017
Summary
Recent research highlights the IL-1 pathway
Area of Science:
- Immunology
- Genetics
- Molecular Biology
- Environmental Science
Background:
- Autoinflammatory bone diseases, including chronic recurrent multifocal osteomyelitis (CRMO), involve complex genetic, molecular, immunologic, and environmental factors.
- Understanding the pathogenesis of both syndromic and non-syndromic CRMO is crucial for developing effective treatments.
Purpose of the Study:
- To review recent advances in understanding the factors contributing to the pathogenesis of autoinflammatory bone diseases.
- To focus on chronic recurrent multifocal osteomyelitis (CRMO), encompassing its syndromic and non-syndromic forms.
Main Methods:
- Review of recent scientific literature on autoinflammatory bone diseases.
- Analysis of genetic, molecular, immunologic, and environmental data.
- Investigation of the IL-1 pathway and its role in CRMO pathogenesis.
Main Results:
- Growing evidence implicates the IL-1 pathway in Mendelian CRMO.
- LIPIN2 regulates the NLRP3 inflammasome via P2X7 receptor activation; cholesterol modulates P2X7R.
- Dietary changes in mouse models alter the microbiome, protecting against sterile osteomyelitis.
- The IL-1 pathway is central to syndromic autoinflammatory bone disorders; lipids and microbiome implicated in sterile osteomyelitis.
Conclusions:
- The IL-1 pathway is a key player in the pathogenesis of syndromic autoinflammatory bone diseases.
- Lipids and the microbiome are emerging factors in sterile osteomyelitis.
- Further research is needed to fully elucidate the genetic and immunologic basis of non-syndromic CRMO.