Stimulated monocytes secreted more IL-18 and IL-1β than controlsMonocytes from patients carrying two MEFV mutations displayed an increased pyrin inflammasome response compared with monocytes from patients with a single mutationMEFV mutations decreased the activation threshold of pyrin inflammasome which might explain the increased release of IL-18 and IL-1β in FMF patients. Here, MEFV is linked to familial Mediterranean fever.