Abstract

Paediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (PIMS-TS) is a new systemic inflammatory disease that mainly affects children. Its course in many features resembles that of acute rheumatic fever (ARF). Therefore, it is interesting that the experiences with ARF can be used in the management of patients with PIMS-TS. The aim of the article is to analyse the current data on PIMS-TS in relation to ARF. PIMS-TS and ARF are associated with an abnormal immune response to specific pathogens (SARS-CoV-2 and group A streptococcus, respectively). The main symptoms of both diseases are fever and cardiac involvement. Current therapy for PIMS-TS is based on anti-inflammatory treatment: intravenous immunoglobulin (first-line), intravenous glucocorticoids (second-line), or biological therapy (third-line; including interleukin [IL]-1 antagonists, IL-6 receptor blockers, and anti-tumour necrosis factor agents). Vaccination might be good prophylaxis, but the efficacy and safety of the vaccines against SARS-CoV-2 have not yet been established in children. Interesting insights may be gained by considering PIMS-TS in light of what is known of ARF due to their similar courses, but there are still many unanswered questions surrounding this disease and its pathogenesis.

Highlights

  • Paediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (PIMS-TS) is a new systemic inflammatory acute onset disease that mainly affects children

  • * diagnosis criterion; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2; paediatric inflammatory multisystemic syndrome (PIMS)-TS—paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2; ARF, acute rheumatic fever; GAS, group A streptococcus; anti-streptolysin O (ASO), Anti-streptolysin O; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; PCT, procalcitonin; IL, interleukin; TNF, tumor necrosis factor; IVIG, intravenous immunoglobulin

  • These results suggest that the inflammatory innate immune response and autoreactivity secondary to SARS-CoV-2 infection may be critical to the pathogenesis of PIMS-TS

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Summary

Introduction

Paediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (PIMS-TS) is a new systemic inflammatory acute onset disease that mainly affects children. The main symptom of PIMS-TS is fever, but its course may be similar to that of other autoinflammatory diseases, such as complete, incomplete, and atypical Kawasaki disease, toxic shock syndrome, sepsis, hemophagocytic syndrome [5] or systemic onset juvenile idiopathic arthritis. None of these other diseases have been linked to one specific pathogen. * diagnosis criterion; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2; PIMS-TS—paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2; ARF, acute rheumatic fever; GAS, group A streptococcus; ASO, Anti-streptolysin O; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; PCT, procalcitonin; IL, interleukin; TNF, tumor necrosis factor; IVIG, intravenous immunoglobulin. Can our medical knowledge with ARF help in understanding PIMS-TS? The aim of this article is to analyse the current data on PIMS-TS in relation to ARF

Relationship with Infection
Clinical Symptoms
Pathogenesis
Genetic Factors
The Role of Inflammatory Cytokines
Immune Cell Activation
Autoantibodies Target Organ Systems Central to PIMS-TS and ARF Pathology
Treatment
Vaccination as Prophylaxis
Conclusions
Findings
Multisystem Inflammatory Syndrome in Children and Adolescents with COVID-19
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