To date, 115 pathogenic/likely pathogenic NLRP3 variants have been reported in patients with CAPS on infevers database (https://infevers.umai-montpellier.fr/web; accessed on 12/19/2021), and most of them affect the NOD domain of the NLRP3 protein. The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.