Overall, the combined AOSD cohort had significantly higher levels of ASC/NLRP3 specks (236.5 [56.2–1,693.0] events/μL, median [interquartile range (IQR)]) compared with HCs (P = 0.0001, 48.1 [22.9–79.1] events/μL) and cases of familial Mediterranean fever (FMF) (P < 0.0001, 31.6 [15.7–73.5] events/μL). This evidence concerns the gene NLRP3 and familial Mediterranean fever.