Childhood Multisystem Inflammatory Syndrome: An Emerging Disease with Prominent Cardiovascular Involvement—A Scoping Review

Abstract

Multisystem inflammatory syndrome in children (MIS-C) or paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is an emerging disease in children affected with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and thought to be an immune-mediated post-infectious complication of SARS-CoV-2. The disease presentation is similar to Kawasaki disease but has certain distinguishing features. The exact pathogenesis is still not clear but an aberrant immune response, antibody-mediated vascular damage and virus-mediated abnormal type I and III interferon-gamma response are thought to be responsible. Most children who are previously healthy present after 2–4 weeks of SARS-CoV-2 infections with febrile illness of short duration with prominent gastrointestinal, cardiac and hematologic manifestations, progressing to vasoplegic shock, requiring vasopressor therapy. Cardiovascular involvement is prominently marked by acute myocardial injury/myocarditis and the development of coronary artery aneurysms. Laboratory markers of inflammation are elevated uniformly. Most children require intensive care, and few need invasive ventilation. The treatment mainly consists of anti-inflammatory and immunomodulatory therapy like intravenous immunoglobulins and steroids. The overall prognosis is good and reported mortality rates are 0–4%.