Multisystem inflammatory syndrome in children associated with COVID-19: from pathophysiology to clinical management and outcomes

Abstract

Multisystem inflammatory syndrome in children (MIS-C), also known as pediatric inflammatory multisystem syndrome (PIMS), is a new postinfectious illness associated with COVID-19, affecting children after SARS-CoV-2 exposure. The hallmarks of this disorder are hyperinflammation and multisystem involvement, with gastrointestinal, cardiac, mucocutaneous, and hematologic disturbances seen most commonly. Cardiovascular involvement includes cardiogenic shock, ventricular dysfunction, coronary artery abnormalities, and myocarditis. Now entering the fourth year of the pandemic, clinicians have gained some familiarity with the clinical presentation, initial diagnosis, cardiac evaluation, and treatment of MIS-C. This has led to an updated definition from the Centers for Disease Control and Prevention in the USA driven by increased experience and clinical expertise. Furthermore, the available evidence established expert consensus treatment recommendations supporting a combination of immunoglobulin and steroids. However, the pathophysiology of the disorder and answers to what causes this remain under investigation. Fortunately, long-term outcomes continue to look promising, although continued follow-up is still needed. Recently, COVID-19 mRNA vaccination is reported to be associated with reduced risk of MIS-C, while further studies are warranted to understand the impact of COVID-19 vaccines on MIS-C. We review the findings and current literature on MIS-C, including pathophysiology, clinical features, evaluation, management, and medium- to long-term follow-up outcomes.