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. 2024 Sep 3;12(9):2014.
doi: 10.3390/biomedicines12092014.

Kawasaki Disease Diagnosis and Treatment in over 1000 Patients: A Continuum of Dysregulated Inflammatory Responses

Stejara A Netea  1   2 , Giske Biesbroek  1 , Diana van Stijn  1 , Sietse Q Nagelkerke  1   3 , Kawasaki Study Group, Cahal Group, Kiri Group, Irene M Kuipers  4 , Taco W Kuijpers  1   2   3
Affiliations

Kawasaki Disease Diagnosis and Treatment in over 1000 Patients: A Continuum of Dysregulated Inflammatory Responses

Stejara A Netea et al. Biomedicines. .

Abstract

Background: Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C).

Methods: A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included. Clinical characteristics were described and risk factors for CAA (persistence) were investigated. Phenotypic patterns of the prospectively included KD patients were evaluated. These patterns were also compared to the seronegative KD and seropositive MIS-C cases identified during the SARS-CoV-2 pandemic.

Results: KD mostly affected boys and children < 5 years. IVIG resistance, CAAs, and giant CAAs occurred in 24.5%, 21.4%, and 6.6%, respectively. Giant CAAs were significantly more likely to normalize to a normal Z score in patients that were younger than 2.5 years old at the time of initial giant CAA (χ2 test p = 0.02). In our prospective (SARS-CoV-2-seronegative) KD series, there was a diminishing male predominance over time, whereas the proportions of incomplete presentations (p < 0.001) and patients with circulatory shock (p = 0.04) increased since the COVID-19 pandemic. Pre- and post-pandemic KD cases presented with different levels of C-reactive protein, thrombocyte counts, and hemoglobin levels over the years. Compared to pandemic KD, SARS-CoV-2-seropositive MIS-C patients were older (p < 0.001), and more often required intensive care admission (p < 0.001), with a gradual decrease over time between 2020 and 2022 (p = 0.04). KD carried a substantial risk of CAA development in contrast to MIS-C.

Conclusion: the phenotypic changes seen over the last twelve years of our prospective follow-up study suggest a spectrum of hyperinflammatory states with potentially different triggering events within this clinical entity.

Keywords: KD; Kawasaki disease; MIS-C; epidemiology; hyperinflammation; mucocutaneous lymph node syndrome; multisystem inflammatory syndrome in children; phenotypic patterns.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Inclusion flowchart.
Figure 2
Figure 2
Number of complete KD, incomplete KD and MIS-C inclusions in the Amsterdam cohort in the COVID-19 pandemic with the dominating strains of SARS-CoV-2 depicted in the dashed frames shown per month of inclusion (A) and continuously over-time (B).
Figure 3
Figure 3
Annual trends seen for male sex distribution (A), complete presentations (B) and circulatory shock (C) in prospectively included patients (all patients included between 2012 and 2023).
See this image and copyright information in PMC

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