Fig. 1.

IVIG-resistant systemic inflammation in KD patients is hallmarked by specific innate immune mediators. A Schematic representation of the KAWAKINRA study protocol indicating sampling at screening visit (prior to anakinra). B Fold change (on MFI level) of blood biomarkers assessed in the present study (n = 23) over pediatric healthy control median. C Multiple correlation analyses of blood biomarkers, clinical markers of inflammation, and circulating cell counts. Black squares are used to highlight prominent clusters of association, and color coding reflects Spearman correlation coefficient. D Ward’s unsupervised hierarchical clustering of serum biomarker levels quantified at screening visit. Color coding indicates Z score. E, F Serum biomarkers (E) and inflammatory parameters (F) reflecting the clustering of patients. Data were analyzed by Kruskal–Wallis followed by Dunn’s multiple comparison (E) or Mann–Whitney U test (F). * = p < 0.05; ** = p < 0.01. ^§Patient 8 received steroids prior to anakinra; *patients 7 and 14 received two cycles of IVIG prior to anakinra. No screening visit serum sample was available for patient 1