The DIRA syndrome, in which endogenous IL-1Ra is lacking, is similar to syndromes of enhanced IL-1β production due to genetic alterations of the cryopyrin gene (Cryopyrin-associated periodic syndromes [CAPS]), which are also responsive to exogenous IL-1Ra [20], thus confirming the key role of IL-1/IL-1Ra balance in disease activity. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.