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/Negative | OR | Sensitivity | Specificity | Accuracy | AUC | Patients not fulfilling the criteria | Number of patients not fulfilling the clinical criteria but satisfying the clinical/genetic criteria | |
| CAPS clinical + genetics | TP: 98/1013 | 4490 | 0.72 | 1 | 0.96 | 0.86 | 27/38 pts, score 0 (71.05%) | |
| TN: 877/1013 | 10/38 pts, score 1 (26.32%) | |||||||
| FP: 0/1013 | 1/38 pts, score 2 (2.63%) | |||||||
| FN: 38/1013 | ||||||||
| CAPS clinical | TP: 82/925 | 220.3 | 0.77 | 0.99 | 0.96 | 0.88 | 3/25 pts, score 0 (13.04%) | Score 0: 0/3 |
| TN: 806/925 | 8/25 pts, score 1 (34.78%) | Score 1: 1/8 | ||||||
| FP: 12/925 | 12/25 pts, score 2 (52.17 %) | Score 2: 11/12 | ||||||
| FN: 25/925 | ||||||||
| FMF clinical + genetics | TP: 304/1010 | 1725.3 | 0.89 | 1 | 0.96 | 0.94 | 12/39 pts, score 0 (30.77%) | |
| TN: 664/1010 | 26/39 pts, score 1 (66.67%) | |||||||
| FP: 3/1010 | 1/39 pts, score 2 (2.56%) | |||||||
| FN: 39/1010 | ||||||||
| FMF clinical | 82.4 | 0.85 | 0.94 | 0.91 | 0.89 | 3/50 pts, score 0 (6,12%) | Score 0: 0/3 | |
| TP: 283/940 | 26/50 pts, score 1 (53,06%) | Score 1: 8/26 | ||||||
| TN: 568/940 | 20/50 pts, score 2 (40,82 %) | Score 2: 19/20 | ||||||
| FP: 39/940 | ||||||||
| FN: 50/940 | ||||||||
| MKD clinical + genetics | 5209.1 | 0.74 | 1 | 0.98 | 0.87 | 2/16 pts, score 0 (12.5%) | ||
| TP: 45/1005 | 14/16 pts, score 1 (87.5%) | |||||||
| TN: 944/1005 | ||||||||
| FP: 0/1005 | ||||||||
| FN: 16/1005 | ||||||||
| MKD clinical | 13.2 | 0.75 | 0.81 | 0.81 | 0.78 | 2/14 pts, score 1 (15.38%) | Score 1: 0/2 | |
| TP: 43/818 | 11/14 pts, score 2 (84.62%) | Score 2: 10/11 | ||||||
| TN: 617/818 | ||||||||
| FP: 144/818 | ||||||||
| FN: 14/818 | ||||||||
| TRAPS clinical + genetics | 2526.9 | 0.74 | 1 | 0.97 | 0.87 | 6/26 pts, score 0 (23.08%) | ||
| TP: 73/1000 | 20/26 pts, score 1 (76.92%) | |||||||
| TN: 900/1000 | ||||||||
| FP: 1/1000 | ||||||||
| FN: 26/1000 | ||||||||
| TRAPS clinical | 30.4 | 0.55 | 0.96 | 0.92 | 0.76 | 27/42 pts, score 1 (71.05%) | Score 1: 8/27 | |
| TP: 52/940 | 11/42 pts, score 2 (28.95 %) | Score 2: 11/11 | ||||||
| TN: 813/940 | ||||||||
| FP: 33/940 | ||||||||
| FN: 42/940 | ||||||||
| PFAPA clinical | TP: 149/1001 | 61.4 | 0.66 | 0.97 | 0.9 | 0.82 | ||
| 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;