Abstract

Background: Comparisons between Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) have been critiqued, with suggestions that KD Shock Syndrome (KDSS) patients may be a more valid comparison. We compared contemporaneous KD and MIS-C patients, both with and without shock at presentation. Methods: The International KD Registry enrolled 2144 patients with either KD (site diagnosis confirmed by AHA criteria) or MIS-C (site diagnosis confirmed by CDC criteria) from 40 sites in 7 countries from 01/2020 to 01/2023. Data collected included demographics, clinical features and presentation, management, laboratory values, and outcomes, and diagnosis/shock groups were compared. Results: Shock at presentation was noted for 19 of 672 (2.8%) KD patients and 653 of 1472 (38%; p<0.001) MIS-C patients. For both groups, shock patients were significantly more likely to be admitted to ICU, receive inotropes, and have cardiac arrest or arrhythmia. Groups did not differ by sex, but MIS-C patients and shock patients were older. While MIS-C shock patients were less likely to be White, KD shock and MIS-C shock patients more likely to be Black. For inflammatory markers, while shock patients in both groups had higher CRP and ferritin compared to non-shock patients, ESR was similar in both groups to non-shock patients, and higher WBC was only noted for MIS-C shock versus MIS-C non-shock patients. KD shock patients had similar values to MIS-C shock patients, with the exception of lower ferritin. Shock patients had higher NTproBNP (both diagnoses) and Troponin I (MIS-C only). Shock patients had lower LV ejection fraction, more so for MIS-C patients ( Figure A ). KD shock patients had higher max coronary artery Z scores versus MIS-C patients ( Figure B ). Conclusions: KD shock patients are more similar to MIS-C patients, particularly those with shock. Shock was associated with worse coronary artery involvement for KD patients, and worse LV dysfunction for both MIS-C and KD.

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