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

Roberta Scianaro1, Antonella Insalaco1, Luisa Bracci Laudiero1

  • 1Rheumatology Unit, Bambino Gesù Children's Hospital, Rome, Italy.

Pediatric Rheumatology Online Journal
|July 26, 2014
PubMed
Abstract

Insights

Chronic recurrent multifocal osteomyelitis (CRMO) involves inflammasome activation. Elevated Interleukin-1 beta (IL-1β) in peripheral blood mononuclear cells (PBMCs) and bone suggests its role in CRMO.

Area of Science:

  • Immunology
  • Rheumatology
  • Molecular Biology

Background:

  • Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory bone disease.
  • The role of inflammasomes in CRMO pathogenesis remains incompletely understood.

Purpose of the Study:

  • To investigate inflammasome component expression in peripheral blood mononuclear cells (PBMCs) and bone biopsies of CRMO patients.
  • To analyze the Interleukin-1 beta (IL-1β) pathway in CRMO.

Main Methods:

  • Quantitative real-time PCR for inflammasome mRNA in PBMCs from 15 CRMO patients and 13 controls.
  • ELISA for IL-1β release from stimulated PBMCs.
  • Immunohistochemistry for inflammasome components (ASC, CASP-1, NLRP3, IL-1β) in CRMO bone biopsies.

Main Results:

  • Significantly higher mRNA levels of ASC, CASP-1, and IL-1β in PBMCs from CRMO patients (active and remission) compared to controls.
  • Increased IL-1β release from LPS-stimulated PBMCs of CRMO patients.
  • Inflammasome components detected in CRMO osteoclasts within bone tissue.

Conclusions:

  • Abnormal regulation of the IL-1β axis is implicated in CRMO pathogenesis.
  • Inflammasome activation in PBMCs and bone may contribute to CRMO.
Keywords:
Autoinflammatory disordersChronic recurrent multifocal osteomyelitisInflammasomeInterleukin-1βOsteoclasts

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