Drugs that block the IL-1 pathway, such as the recombinant IL-1Ra anakinra, the soluble decoy receptor rilonacept, and the anti-IL-1β monoclonal antibody canakinumab, induce rapid and sustained remission across a spectrum of diseases, from monogenic syndromes like CAPS and DIRA to polygenic conditions like systemic juvenile idiopathic arthritis and gout (14). Here, IL1B is linked to cryopyrin-associated periodic syndrome.