Table 4

Performance of the new classification criteria to discriminate different recurrent fevers in the validation data set of patients extracted from the Eurofever Registry (N=1018)

Positive/NegativeORSensitivitySpecificityAccuracyAUCPatients not fulfilling the criteriaNumber of patients not fulfilling the clinical criteria but satisfying the clinical/genetic criteria
CAPS clinical + geneticsTP: 98/101344900.7210.960.8627/38 pts, score 0 (71.05%)
TN: 877/101310/38 pts, score 1 (26.32%)
FP: 0/10131/38 pts, score 2 (2.63%)
FN: 38/1013
CAPS clinicalTP: 82/925220.30.770.990.960.883/25 pts, score 0 (13.04%)Score 0: 0/3
TN: 806/9258/25 pts, score 1 (34.78%)Score 1: 1/8
FP: 12/92512/25 pts, score 2 (52.17 %)Score 2: 11/12
FN: 25/925
FMF clinical + geneticsTP: 304/10101725.30.8910.960.9412/39 pts, score 0 (30.77%)
TN: 664/101026/39 pts, score 1 (66.67%)
FP: 3/10101/39 pts, score 2 (2.56%)
FN: 39/1010
FMF clinical82.40.850.940.910.893/50 pts, score 0 (6,12%)Score 0: 0/3
TP: 283/94026/50 pts, score 1 (53,06%)Score 1: 8/26
TN: 568/94020/50 pts, score 2 (40,82 %)Score 2: 19/20
FP: 39/940
FN: 50/940
MKD clinical + genetics5209.10.7410.980.872/16 pts, score 0 (12.5%)
TP: 45/100514/16 pts, score 1 (87.5%)
TN: 944/1005
FP: 0/1005
FN: 16/1005
MKD clinical13.20.750.810.810.782/14 pts, score 1 (15.38%)Score 1: 0/2
TP: 43/81811/14 pts, score 2 (84.62%)Score 2: 10/11
TN: 617/818
FP: 144/818
FN: 14/818
TRAPS clinical + genetics2526.90.7410.970.876/26 pts, score 0 (23.08%)
TP: 73/100020/26 pts, score 1 (76.92%)
TN: 900/1000
FP: 1/1000
FN: 26/1000
TRAPS clinical30.40.550.960.920.7627/42 pts, score 1 (71.05%)Score 1: 8/27
TP: 52/94011/42 pts, score 2 (28.95 %)Score 2: 11/11
TN: 813/940
FP: 33/940
FN: 42/940
PFAPA clinicalTP: 149/100161.40.660.970.90.82
TN: 752/1001
FP: 24/1001
  • For explanation of the scores 0–1–2, see online supplementary table 6.

  • CAPS, cryopyrin-associated periodic syndromes; FMF, familial Mediterranean fever; FN, false negative; FP, false positive; MKD, mevalonate kinase deficiency; PFAPA, periodic fever, aphthosis, pharyngitis and adenitis; pts, patients.TN, true negative; TP, true positive; TRAPS, tumour necrosis factor receptor-associated periodic fever syndrome;