Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Clin Immunol. 2015 Oct;160(2):277-81. doi: 10.1016/j.clim.2015.06.005. Epub 2015 Jun 20.


To assess the role of IL-6/IL-18 in the pathogenesis of systemic juvenile idiopathic arthritis (s-JIA) and to investigate the clinical significance of serum IL-18 levels for predicting macrophage activation syndrome (MAS) development, we measured the serum IL-6/IL-18 levels in 76s-JIA patients, including 15 with MAS, and compared them with the clinical features. We identified 2 distinct subsets on the basis of serum IL-6/IL-18 levels. The IL-18-dominant subset had more patients who developed MAS. Serum IL-18 levels during active phase in patients with MAS were significantly higher than those without MAS. The cutoff value of serum IL-18 levels for predicting MAS development was 47750 pg/ml. The patients with IL-18 dominant subset at their disease onset were significantly more likely to develop MAS after TCZ therapy started. IL-18 might have a key role in the pathogenesis of MAS. Serum IL-18 levels >47750 pg/ml might be useful to predict MAS development.

Keywords: Interleukin-18 (IL-18); Prediction of macrophage activation syndrome; Systemic juvenile idiopathic arthritis.

MeSH terms

  • Adolescent
  • Arthritis, Juvenile / immunology*
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Cohort Studies
  • Female
  • Humans
  • Interleukin-18 / immunology*
  • Interleukin-6 / immunology*
  • Longitudinal Studies
  • Macrophage Activation Syndrome / immunology*
  • Male
  • Prognosis


  • IL6 protein, human
  • Interleukin-18
  • Interleukin-6