S100A8 /S100A9

1. SJIA SJIA F Q KD FI F Q KD FI Relative DIGE MEASUREMENTS Relative ELISA MEASUREMENTS A2M APOA1 CRP HP S100A9 S100A8 SAA SAP A2M APOA1 CRP HP S100A8 /S100A9 SAA SAP Figure 4

2. C A B D Training set n = 24 Testing set n = 20 Training Testing SJIA • Biomarker panel • of 7 members • A2M • APO AI • CRP • HP • S100A8/S100A9 • SAA • SAP SJIA Clinical diagnosis Clinical diagnosis SJIA F SJIA Q SJIA F SJIA Q F Q F Q 12 12 10 10 n = n = LDA Testing Classified as F Classified as F 11 1 8 3 Predicted probabilities Classified as Q Classified as Q 1 11 2 7 91.6% 91.6% 80% 70% Percent Agreement with clinical diagnosis + - Percent Agreement with clinical diagnosis + - 91.6% 75% Overall P = 1.0 E -4 Overall P = 7 E -2 Patient samples P*= 9.6E -6 P*=4 E -3 Figure 5

3. E Training Set Testing Set Training + Testing Set Sensitivity Sensitivity Sensitivity ESR : AUC=0.86 ESR : AUC=0.96 ESR : AUC=0.92 S100A8/S100A9 : AUC=0.78 S100A8/S100A9 : AUC=0.73 S100A8/S100A9 : AUC=0.74 CRP : AUC=0.65 CRP : AUC=0.82 CRP : AUC=0.72 panel : AUC=0.82 panel : AUC=0.95 panel : AUC=0.94 1- Specificity 1- Specificity 1- Specificity Figure 5

4. D B C A Training set n = 23 Testing set n = 15 Training Testing POLY F vs. Q POLY POLY Clinical diagnosis Clinical diagnosis PF PQ PF PQ F Q F Q 13 10 10 5 n = n = Sensitivity LDA Testing Classified as F Classified as F 10 5 3 0 Predicted probabilities Classified as Q Classified as Q 3 5 7 5 76.9% 50% 30% 100% Percent Agreement with clinical diagnosis + - Percent Agreement with clinical diagnosis + - panel : AUC=0.64 65.2% 53.3% CRP : AUC=0.64 Overall P = 0.41 Overall P = 0.20 Patient samples 1- Specificity Supplementary Figure 4

5. SJIA F KD FI SJIA F Data set n = 34 KD FI 10 12 12 SJIA F NOT-SJIA F Clinical diagnosis 10 24 n = SAP Unsupervised clustering SAA S100A8 S100A9 Clustered as SJIA F 7 1 Clustered as NOT-SJIA F 3 23 HP 70% 95.8% CRP - Percent Agreement with clinical diagnosis + 88.2% APOA1 Overall P = 1.6 E -4 A2M Supplementary Figure 5

6. A. B KD + FI Training set n = 59 SJIA F KD FI SJIA F KD + FI 22 10 27 SJIA F NOT SJIA F Clinical diagnosis Sensitivity 22 37 n = ESR : AUC=0.661 LDA Predicted probabilities S100A8/S100A9 : AUC=0.543 Classified as SJIA F 16 3 CRP : AUC=0.627 6 34 Classified as NOT SJIA F 72.8% 91.9% panel : AUC=0.801 Percent Agreement with clinical diagnosis + - 84.7% Overall P = 4.3E -7 1- Specificity Patient samples Figure 6

7. C B A QF QQ SJIA Clinical diagnosis QF QQ 5 10 Sensitivity n = LDA Predicted probabilities Classified as QF 4 0 Classified as QQ 1 10 ESR : AUC=0.68 80% 100% S100A8/S100A9 : AUC=0.74 Percent Agreement with clinical diagnosis + - CRP : AUC=0.82 93.3% panel : AUC=0.90 Overall P = 3.7 E -3 Patient samples 1- Specificity Figure 7

8. Figure 8