Supplement Table 7: Study design and prognostic parameters of the respective score (cursive), including studies evaluating the scores.
Sen = sensitivity, spe = specificity, PPV = Positive Predictive Value, NPV = Negative Predictive Value,
Score Validated through
Subjects included to evaluation
Study design Refractory
Def. (%)
Country Prognostic parameters
Kobayashi Kobayashi et al. 5
N=546 (development dataset) N=204 (Validation dataset)
Retrospective, monocentric data to develop the score and prospective for validation
Fever >24h (21%)
Japan IVIG-Resistance:
Sen 86%, Spe 68%
Seki et al.18
N=1626
N=990: IVIG 1g in 2 doses (A) N=636: IVIG 2 g in 1 dose (B)
Retrospective, multicentric, Comparison between IVIG 1g in 2 doses vs. 2g in 1 dose
Fever >24 h (22,9% A, 22,5%
B)
Japan IVIG resistance: A: CAA at 4 weeks A:
Sen 74%, Spe 79% Sen 83%, Spe 68%
IVIG resistance B: CAA at 4 weeks B:
Sen 70%, Spe 79% Sen 80%, Spe 71%
Kanamitsu et al.17
N=132 typical KD N=51 incomplete KD
Retrospective, monocentric Comparison between typical KD and incomplete KD Score ≥ 4
2. IVIG therapy (22%, typical KD,17.6%
incomplete KD)
China IVIG resistence:
Typical KD
Sen 79 %, Spe 49 % PPV 30 %, NPV 89 % Inomplete KD
Sen 67 %, Spe 52%, PPV 23 %, NPV 88%
Kim et al.19 N=703 Retrospective review 2. IVIG- therapy,
fever > 48 h (16,7%)
Korea IVIG resistance:
Sen 31%, Spe 87%
Sleeper et al.9
N=62 (patients not treated with steroids)
Data from a randomized trial of pulsed steroids for primary KD treatment
Score ≥ 4
Fever >36 h (14,5%)
USA IVIG resistance:
Sen 33%, Spe. 87%
Davies et al.10
N=59 Retrospective monocentric
KS-Score ≥ 4
Fever >24 h (32,2%)
England IVIG resistence:
Sen 58%, Spe 35%, PPV 24%, NPV 73%
CAA (acute):
Sen 60 %, Spe 39%, PPV 32 %, NPV 64%
Sano Sano et
al.8
N=112 Retrospective Fever >24 h
(19,6%)
Japan IVIG resistance:
Sen 77%, Spez 86%, PPV 59%, NPV 94%
Kanamitsu et al.17
N=119 typical KD N=44 incomplete KD
See Kobayashi See Kobayashi China IVIG resistance:
Typical KD
Sen 72%, Spe 68%, PPV 39%, NPV 88%
Incomplete KD
Sen 36%, Spe 85%, PPV 43%, NPV 89%
Kim et al.19 N=703 See Kobayashi See Kobayashi Korea IVIG resistance:
Sen 33%, Spe 87%
Sleeper et al.9
N=56 (patients not treated with steroids)
See Kobayashi See Kobayashi USA IVIG resistance:
Sen. 42%, Spe. 87%
Egami Egami et al.7
N=320 Retrospective Fever and
CRP↓<50%
<48h (13%)
Japan IVIG resistance:
Sen 78%, Spez 76%, PPV 32%, NPV 96%
CAA (acute):
Sen 61%, Spe 81%
Kanamitsu et al.17
N=132 typical KD N=51 incomplete KD
See Kobayashi See Kobayashi China IVIG resistance:
Typical KD
Sen 72%, Spe 68%, PPV 39%, NPV 90%
Inkomplete KD
Sen 56%, Spe 68%, PPV 56%, NPV 87%
Kim et al.
19
N=703 See Kobayashi See Kobayashi Korea IVIG resistance:
Sen 28%, Spe 91%
Sanchez- Manubens et al.21
N=305 Retrospective 2. Dose of IVIG Spain IVIG resistance:
Sen 26%, Spez 82%
PPV 22%, NPV 85%
Sleeper et al.9
N=78 (patients not treated with steroids)
See Kobayashi Fever > 36h (15,4%)
USA IVIG resistance:
Sen 42%, Spez 85%
