NLRP3 and Autoimmunity: Monogenic autoinflammatory diseases are also characterized by a clinical and biological inflammatory syndrome in which there is little or no evidence of autoimmunity.3 One of the prototypic monogenic autoinflammatory diseases is the cryopyrin-associated periodic syndrome, which is caused by NLRP3 selective gene mutations.4 NDs share many clinical features of monogenic inflammatory disorders, including fever, arthralgia, and neutrophilic infiltration of the skin and visceral organs.