Moreover, Aksentijevich el al. (2009) [38] and Reddy et al. (2009) [39] reported an hitherto unknown association of a severe autoinflammatory syndrome in 10 patients with homozygous mutations of IL1RN, the gene encoding IL-1Ra, leading to the definition of a new syndrome, deficiency of the Interleukin-1 receptor antagonist [DIRA] [3]. Here, IL1RN is linked to autoinflammatory syndrome.