Kawasaki disease in the time of COVID-19-the international kawasaki disease registry

ABSTRACT

Background and Aim: The considerable overlap in case definition and clinical features between patients with COVID-19 associated Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki disease (KD) suggests shared pathogenesis. We sought to compare demographic, clinical presentation, management and outcomes of patients by COVID-19 status. Method(s) The International KD Registry (IKDR) began enrolling patients with clinical features of either acute MIS-C or KD or fever with hyperinflammation beginning in January 2020. The IKDR is unique regarding broad patient selection and includes sites from North, Central and South America, Europe, Asia and the Middle East. Patient groups stratified by COVID-19 status were compared. Result(s) As of October 6, 2021, 1330 patients were registered from 31 sites. COVID status was POSITIVE for 59% (confirmed household COVID-19 contact and/or positive SARS-CoV-2 PCR or serology), POSSIBLE for 4% (suggestive clinical features but some negative tests or absent exposure), NEGATIVE for 23%, and UNKNOWN (no known exposure and testing not com-pleted) for 14% (TABLE). Most of the UNKNOWN patients were from early in the COVID-19 pandemic before MIS-C was defined and before COVID-19 serologic testing was widely used. POSITIVE and POSSIBLE patients were older, had fewer KD clinical criteria, greater gastrointestinal symptoms, were more likely to present with shock and require ICU admission and inotropic support. POSSIBLE patients had greater days from symptom onset to first immune modulation treatment, with no differences between groups regarding days from admission to first treatment. Most patients in each group received intravenous immune globu-lin, with POSITIVE and POSSIBLE patients more likely to have received steroids and anakinra. NEGATIVE and UNKNOWN patients had higher maximal coronary artery Z scores, with a trend to having higher categories of aneurysm involvement. Conclusion(s) While there was considerable overlap in presentation, management and outcomes between COVID-19 POSITIVE/POSSIBLE (presumed MIS-C) and COVID NEGATIVE/UNKNOWN patients (presumed KD), COVID-19 POSITIVE/POSSIBLE patients had more severe presentations and required more intensive management, although coronary artery outcomes trended to be less severe. Patient recruitment con-tinues, and in-depth comparison of laboratory features and appli-cation of machine learning approaches to patient differentiation and prediction of optimal management pathways are forthcoming.