IL1R1 and cryopyrin-associated periodic syndrome: Patients with CAPS exhibit extremely positive responses to all commercially available IL-1 inhibitors, including the blockade of IL-1α and IL-1β and the endogenous IL-1Ra at the IL-1R1 level (anakinra) (15–17) or in the extracellular space (rilonacept) (22, 23) or by using a monoclonal antibody (MoAb) that specifically binds IL-1β in the extracellular milieu (canakinumab) (24, 25).