Interestingly, homozygous NLRC4V341A/V341A mice recapitulated elevated serum IL-18 levels as in AIFEC patients (4–7), although the IL-18 serum levels observed in NLRC4V341A/V341A mice were much lower than observed in NLRC4V341A-expressing AIFEC patients (4, 5). Here, IL18 is linked to periodic fever-infantile enterocolitis-autoinflammatory syndrome.