Deregulation of the IL-1β axis in chronic recurrent multifocal osteomyelitis.

Pediatr Rheumatol Online J 2014 17;12:30. Epub 2014 Jul 17.

Rheumatology Unit, Bambino Gesù Children's Hospital, Rome, Italy.

Background: This study aims to investigate the inflammasome response in peripheral blood mononuclear cells (PBMCs) and the expression of inflammasome components in bone biopsies from patients with chronic recurrent multifocal osteomyelitis (CRMO).

Methods: The expression of inflammasome components mRNAs was evaluated in PBMCs isolated from 15 CRMO patients and 13 healthy controls by quantitative real-time PCR. The Interleukin (IL)-1β released in the medium of PBMC cultures after treatment with lipopolysaccharides (LPS) alone or LPS and ATP was measured by ELISA. Immunohistochemical staining for Apoptosis-associated Speck-like protein (ASC), caspase-1 (CASP-1), Nod-like receptor protein-3 (NLRP3) and IL-1β expression was performed in bone biopsies from CRMO patients.

Results: mRNA levels of ASC, CASP-1 and IL-1β were significantly higher in freshly isolated PBMCs from CRMO patients in active disease than in healthy controls. CASP-1 and IL-1β transcript levels were significantly higher also in PBMCs from CRMO patients in remission compared to healthy controls. PBMCs from CRMO patients in active disease stimulated in vitro with LPS showed a significant increase in IL-1β release compared to healthy control cells. Immunohistochemistry staining of bone tissue revealed the expression of inflammasome components in CRMO osteoclasts.

Conclusions: Our data suggest that an abnormal regulation of IL-1β axis may be involved in CRMO pathogenesis.

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http://dx.doi.org/10.1186/1546-0096-12-30DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4109750PMC
November 2015
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