Cryopyrin-Associated Periodic Syndrome (CAPS), now referred to as NLRP3-associated autoinflammatory disease (NLRP3-AID), was historically described in terms of three distinct clinical entities: Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS) and Chronic Infantile Neurologic Cutaneous Articular syndrome (CINCA), alternatively called Neonatal Onset Multisystem Inflammatory Disease (NOMID). Here, NLRP3 is linked to CINCA syndrome.