Since PFAPA shares clinical similarities with monogenic fever syndromes, several studies have investigated the possible involvement of the genes responsible for Familial Mediterranean Fever (MEFV gene), TNF-Receptor Associated Periodic Syndrome (TNFRSF1A gene), Mevalonate kinase deficiency (MVK gene), and Cryopyrin-Associated Periodic Syndrome (NLRP3 gene) in PFAPA cohorts (15). This evidence concerns the gene TNFRSF1A and PFAPA syndrome.