Although the initial reports of coronavirus disease (COVID-19) cases in children described that children were largely protected from severe manifestations, clusters of pediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection began to be reported in the latter half of April 2020. A novel syndrome called “multisystem inflammatory syndrome in children” (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease (KD), toxic shock syndrome (TSS), and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (MAS). Our objective was to develop a protocol for the evaluation, treatment, and follow-up of patients with MIS-C.
The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of pediatric critical care, cardiology, rheumatology, surgery, gastroenterology, hematology, immunology, infectious disease, and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback.
Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a pediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients’ phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.

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