Table 1.
Summary of the literature on refractory KD in very young infants and the use of infliximab and anakinra in refractory disease.
| Publication | Major findings | Study design | Cohort size | PMID |
|---|---|---|---|---|
| Resistance to standard treatment in very young infants | ||||
| Kobayashi et al. (2006) | Higher risk of developing resistance to IVIG in younger patients, patients with low sodium and platelet count, and patients with high levels of CrP and AST | Retrospective | 546 | 16735679 |
| Mastrangelo et al. (2019) | Very young infants are more likely to have incomplete KD, more likely to show coronary artery involvement and higher resistance to IVIG treatment | Retrospective | 113 | 31493782 |
| Egami et al. (2006) | Younger age, longer illness duration, low platelet count, and high ALT and CrP levels are associated with IVIG-unresponsiveness |
Randomized controlled multicenter trial | 320 | 16887442 |
| Rosenfeld et al. (1995) | Infants younger than 1 year are at greater risk of developing giant CAA |
Retrospective | 443 | 7699529 |
| Kim et al. (2023) | Age under 1 year and resistance to the first IVIG treatment are associated with a higher recurrence rate | Retrospective | 19,456 | 36549869 |
| Garcia et al. (2020) | KD in patients under 3 months of age is more often incomplete and shows a higher risk of cardiac complications | Retrospective | 688 | 32793527 |
| Studies on infliximab for the treatment of KD | ||||
| Burns et al. (2021) | Infliximab might lead to a shorter duration of fever and shorter hospitalization stay for patients with IVIG-resistant KD compared to a second IVIG infusion | Randomized controlled multicenter trial | 105 | 34715057 |
| Masuda et al. (2017) | Infliximab seems to be effective in treating refractory KD in a cohort of 434 patients |
Retrospective | 434 | 29224935 |
| Hur et al. (2019) | Early use of infliximab might reduce the incidence of pronounced CAA in patients with refractory KD | Retrospective | 102 | 30468032 |
| Son et al. (2020) | Infliximab therapy was associated with a shorter fever duration and shorter hospitalization stay | Retrospective | 106 | 21129756 |
| Miyata et al. (2023) | Initial adjunctive therapy with infliximab is associated with a higher CAA regression rate in patients with initial CAAs | Retrospective | 168 | 37258054 |
| Tremoulet et al. (2014) | Addition of infliximab to initial therapy reduced fever duration, inflammation markers, and left anterior descending coronary artery Z-score in KD patients | Randomized controlled phase III trial | 196 | 24572997 |
| Mori et al. (2018) | Higher rate of defervescence in KD patients with initial IVIG resistance receiving infliximab compared to a second IVIG infusion | Randomized controlled phase III trial | 31 | 29386515 |
| Nagatomo et al. (2018) | Infliximab therapy may be effective for the early improvement of CAAs in KD patients with IVIG resistance | Retrospective | 49 | 30144998 |
| Li et al. (2021) | Infliximab is effective for patients with IVIG resistance and leads to a shorter and milder clinical course | Meta-analysis | 33652059 | |
| Studies on anakinra for the treatment of KD | ||||
| Yang et al. (2021) | Anakinra therapy is safe in patients with KD | Randomized controlled phase I/IIa trial | 22 | 34953816 |
| Koné-Paut et al. (2020) | Anakinra may reduce fever duration, inflammation markers, and coronary artery dilation in patients with IVIG-refractory KD | Phase II open-label study | 16 | 32779863 |
| Koné-Paut et al. (2018) | Anakinra used in the late disease course led to reduced inflammation | Retrospective | 11 | 29885546 |