Anakinra, a recombinant IL-1Ra which blocks the activities of the proinflammatory cytokines IL-1α and IL-1β, is clinically used for rheumatoid arthritis (RA), neonatal-onset multisystem inflammatory disease (NOMID), cryopyrin-associated periodic syndromes (CAPS), systemic juvenile idiopathic arthritis (sJIA), adult-onset Still disease (AOSD), Schnitzler’s Syndrome (SS), and deficiency of IL-1RA (DIRA) (15). Here, IL1R1 is linked to CINCA syndrome.