NLRC4 and macrophage activation syndrome: For instance, NLRP1 mutations lead to NLRP1-associated Auto-Inflammation with Arthritis and Dyskeratosis (NAIAD) [12] and NLRC4 gain-of-function mutations triggers Syndrome of enterocolitis and Autoinflammation associated with mutation in NLRC4 (SCAN4) [13] or recurrent Macrophage Activation Syndrome (MAS) [14], (see Table 2).