Bilade Cherqaoui1,2,3, Isabelle Koné-Paut1,2,4, Hélène Yager1, Fleur Le Bourgeois5, Maryam Piram2,4,6. 1. Paediatric rheumatology, APHP, CHU Bicêtre, Le Kremlin-Bicêtre, France. 2. CEREMAIA, French reference centre for auto-inflammatory diseases and inflammatory amyloidosis, CHU Bicêtre, Le Kremlin-Bicêtre, France. 3. University of Paris-Saclay, INSERM, UMR 1173, Infection and inflammation, Montigny-Le-Bretonneux, France. 4. University of Paris-Saclay, France. 5. Paediatric Intensive Care Unit, APHP, CHU Robert Debré, Paris, France. 6. Paediatric Dermatology, CHU Sainte Justine Research Centre, University of Montreal, Montreal, Canada.
Abstract
OBJECTIVE: To better define the clinical distinctions between the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related paediatric inflammatory multi-system syndrome (PIMS) and Kawasaki disease (kDa). METHODS: We compared three groups of patients: group 1, cases from our national historic kDa database (kDa-HIS); group 2, patients with kDa admitted to an intensive care unit (kDa-ICU) from both our original cohort and the literature; and group 3, patients with PIMS from the literature. RESULTS: kDa-HIS included 425 patients (male to female ratio 1.3, mean age 2.8 ± 2.4 years), kDa-ICU 176 (male to female ratio 1.3, mean age 3.5 ± 3.1 years), and PIMS 404 (male to female ratio 1.4, mean age 8.8 ± 3.7 years). As compared with kDa-HIS patients, kDa-ICU and PIMS patients had a higher proportion of cardiac failure and digestive and neurological signs. They also had lower frequency of typical mucocutaneous signs, lower platelet count, higher C-reactive protein level, and lower sodium level. As compared with kDa-HIS and kDa-ICU patients, PIMS patients were older and more frequently had myocarditis. They had fewer coronary abnormalities and lower sodium level. Unresponsiveness to intravenous immunoglobulins was more frequent in kDa-ICU than kDa-HIS and PIMS patients. CONCLUSION: On clinical grounds, regular kDa, kDa-ICU and PIMS might belong to a common spectrum of non-specific pathogen-triggered hyperinflammatory states. The causes of increasing inflammation severity within the three entities and the different effects on the heart remain to be determined.
OBJECTIVE: To better define the clinical distinctions between the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related paediatric inflammatory multi-system syndrome (PIMS) and Kawasaki disease (kDa). METHODS: We compared three groups of patients: group 1, cases from our national historic kDa database (kDa-HIS); group 2, patients with kDa admitted to an intensive care unit (kDa-ICU) from both our original cohort and the literature; and group 3, patients with PIMS from the literature. RESULTS: kDa-HIS included 425 patients (male to female ratio 1.3, mean age 2.8 ± 2.4 years), kDa-ICU 176 (male to female ratio 1.3, mean age 3.5 ± 3.1 years), and PIMS 404 (male to female ratio 1.4, mean age 8.8 ± 3.7 years). As compared with kDa-HISpatients, kDa-ICU and PIMS patients had a higher proportion of cardiac failure and digestive and neurological signs. They also had lower frequency of typical mucocutaneous signs, lower platelet count, higher C-reactive protein level, and lower sodium level. As compared with kDa-HIS and kDa-ICU patients, PIMS patients were older and more frequently had myocarditis. They had fewer coronary abnormalities and lower sodium level. Unresponsiveness to intravenous immunoglobulins was more frequent in kDa-ICU than kDa-HIS and PIMS patients. CONCLUSION: On clinical grounds, regular kDa, kDa-ICU and PIMS might belong to a common spectrum of non-specific pathogen-triggered hyperinflammatory states. The causes of increasing inflammation severity within the three entities and the different effects on the heart remain to be determined.