Although it has been suggested that IL-18 is useful as a biomarker to reflect the therapeutic response and disease activity of AOSD [28, 31, 37] and a phase II clinical trial showed that IL-18 inhibition by administration of recombinant human IL-18BP is effective in the treatment of AOSD [38], further longitudinal studies are needed to verify whether the cytokines identified in this study are associated with therapeutic responses. Here, IL18 is linked to adult-onset Still disease.