Clinical features of CAPS are related to systemic effects of IL-1β-inducing fever, malaise, fatigue, and chronic pain along with a blood serum rise of acute-phase reactants, such as C-reactive protein and serum amyloid A. CINCA/NOMID is characterized for an almost continuous early onset inflammatory state with fever and non-pruritic migratory urticaria-like rash; central nervous system symptoms and arthropathy are common. Here, IL1B is linked to cryopyrin-associated periodic syndrome.