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Published on: November 5, 2021
SARS-CoV-2-Induced Kawasaki-Like Hyperinflammatory Syndrome: A Novel COVID Phenotype in Children
Francesco Licciardi1,2, Giulia Pruccoli3, Marco Denina1,2
1Department of Pediatric and Public Health Sciences, University of Torino, Turin, Italy.
Insights
Two children developed a SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome with fever and diarrhea. This condition, characterized by mucocutaneous signs and capillary leak, suggests a delayed inflammatory response to the virus.
Area of Science:
- Pediatrics
- Infectious Diseases
- Immunology
Background:
- Kawasaki disease is an acute febrile illness affecting infants and young children, primarily characterized by systemic vasculitis.
- The COVID-19 pandemic has been associated with various clinical manifestations in children, including multisystem inflammatory syndrome in children (MIS-C).
- Some MIS-C cases exhibit features overlapping with Kawasaki disease, necessitating further investigation into distinct pathophysiological pathways.
Purpose of the Study:
- To describe a novel hyperinflammatory syndrome in children following SARS-CoV-2 infection.
- To characterize the clinical, laboratory, and immunological features of this SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome.
- To evaluate the potential therapeutic response to intravenous corticosteroids.
Main Methods:
- Case report detailing the clinical presentation and diagnostic workup of two pediatric patients.
- Analysis of clinical signs including persistent fever, profuse diarrhea, and mucocutaneous involvement.
- Laboratory investigations included inflammatory markers, lymphopenia, thrombocytopenia, complement levels, hypoalbuminemia, and serological testing for SARS-CoV-2 (IgM, IgG) and PCR.
Main Results:
- Both patients presented with fever, diarrhea, and mucocutaneous symptoms resembling Kawasaki disease.
- Laboratory findings revealed elevated inflammation markers, lymphopenia, thrombocytopenia, complement consumption, and capillary leak syndrome.
- Despite negative SARS-CoV-2 PCR, high IgM and IgG titers indicated a past infection, suggesting a delayed inflammatory response.
- Intravenous corticosteroid treatment showed positive results.
Conclusions:
- A distinct SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome is proposed, characterized by delayed immune response.
- This syndrome shares similarities with Kawasaki disease and macrophage activation syndrome but has unique features like prodromal diarrhea and capillary leak.
- Early recognition and prompt treatment with corticosteroids may be beneficial for managing this hyperinflammatory condition.
Abstract:
We describe 2 children with persistent fever and profuse diarrhea who developed signs of mucocutaneous involvement (conjunctivitis, fissured lips, skin rash, erythema, and edema of the hands and feet). Blood tests revealed elevated markers of inflammation, lymphopenia, thrombocytopenia, and complement consumption. Afterward, diffuse edema with hypoalbuminemia appeared in the context of a capillary leak syndrome. In both patients, repeated nasal swabs were negative for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), but each patient had high titers of immunoglobulin G and immunoglobulin M against the SARS-CoV-2 virus. The negative PCR results in the presence of immunoglobulin M and immunoglobulin G suggested that the inflammatory response developed in the late phase of viral infection, when SARS-CoV-2 was not detectable in the upper airway. In this report, we describe patients with what we propose to name as SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome. SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome seems to be caused by a delayed response to SARS-CoV-2. It resembles Kawasaki disease complicated by macrophage activation syndrome, although it has peculiar features, such as prodromal diarrhea, capillary leak syndrome, and myocardial dysfunction. Intravenous corticosteroid treatment appears to be helpful.
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