Although no notable mutations in NLRP3 have been identified in sJIA patients, synovial fluid and sera contain elevated levels of IL-1β and IL-18, which are both dependent on NLRP3 inflammasome assembly and may indicate an increased activation of the inflammasome and enhanced caspase-1 activity [94]. This evidence concerns the gene NLRP3 and systemic-onset juvenile idiopathic arthritis.