COVID-19 and Hyperinflammatory Syndrome in Children: Kawasaki Disease with Macrophage Activation Syndrome in Disguise?

Rohit S Loomba,Enrique G Villarreal,Saul Flores

doi:10.7759/cureus.9515

Abstract

A hyperinflammatory syndrome has been described in times of COVID-19 in children. In the setting of uncertainty due to a new virus, the so-called hyperinflammatory syndrome has been coined as a novel entity by some and is being referred to as pediatric inflammatory multisystem syndrome (PIMS). However, the characteristics of the syndrome resemble those of Kawasaki disease (KD), an inflammatory syndrome in children that can lead to coronary artery abnormalities due to a subsequent vasculitis. Furthermore, Kawasaki disease may occasionally trigger macrophage activation syndrome (MAS), a condition in which there is uncontrolled activation and proliferation of macrophages and other cell types, and could lead to multiorgan system dysfunction. This study provides a review of the data regarding COVID-19, Kawasaki disease, and macrophage activation syndrome to demonstrate the similarities and differences between the inflammatory syndrome seen with COVID-19 and KD. In addition, a framework for diagnosis and evaluation is provided that focuses on the pathway previously established for KD and MAS. The authors believe that based on current knowledge, KD treatment delays may carry deleterious effects in the near future for children with COVID-19-related Kawasaki disease.

Highlights

BackgroundAs the COVID-19 pandemic continues globally, a hyperinflammatory syndrome in children has been described [1,2]
The characteristics of the syndrome resemble those of Kawasaki disease (KD), an inflammatory syndrome in children that can lead to coronary artery abnormalities due to a subsequent vasculitis
This study provides a review of the data regarding COVID-19, Kawasaki disease, and macrophage activation syndrome to demonstrate the similarities and differences between the inflammatory syndrome seen with COVID-19 and KD

Summary

Introduction

As the COVID-19 pandemic continues globally, a hyperinflammatory syndrome in children has been described [1,2]. KD, in both its typical and atypical presentations, has been described to be associated with viral illness, and in up to 2% of children, KD can be associated with macrophage activation syndrome (MAS) [3]. A potential pathway by which severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infects cells requires the angiotensin-converting enzyme 2 (ACE2) receptors and triggers pyroptosis and apoptosis resulting in the release of adenosine triphosphate and nucleic acids [12] This leads to CD4+ T lymphocytes activating into pathogenic T-helper cells [13]. The difference for the mortality rate difference remains unclear, patients with JIA tend to be chronically ill for longer periods of time and may carry other comorbidities as compared to children with KD

Discussion

Conclusions

Findings

Disclosures