Due to the enormous interest raised by the identification of NLRP3 Inflammasome (2) and by the dramatic response to interleukin-1 (IL-1) blockers observed in patients presenting a gain of function mutation of NLRP3 gene (cryopyrin-associated periodic syndromes, CAPSs) (3–5), IL-1, is now considered the pivotal pro-inflammatory cytokine in these disorders. Here, IL1A is linked to cryopyrin-associated periodic syndrome.