As for the pathophysiological role of NLRC4, clinical studies have shown that gain‐of‐function NLRC4 mutations are associated with autoinflammation with infantile enterocolitis (AIFEC) (Canna et al, 2014; Romberg et al, 2014), an extremely rare disease that is characterized by macrophage activation syndrome and severe inflammation of gastrointestinal tract. This evidence concerns the gene NLRC4 and periodic fever-infantile enterocolitis-autoinflammatory syndrome.