Delineating phenotypes of Kawasaki disease and SARS-CoV-2-related inflammatory multisystem syndrome: a French study and literature review

• Published in: Rheumatology Vol. 60; no. 10; pp. 4530 - 4537
• Main Authors: Cherqaoui, Bilade, Koné-Paut, Isabelle, Yager, Hélène, Bourgeois, Fleur Le, Piram, Maryam
• Format: Journal Article Web Resource
• Language: English
• Published: England Oxford University Press 01.10.2021; Oxford University Press (OUP)
• Subjects: Ecology, environment; Health; Life Sciences; Original; systemic vasculitis; COVID-19; Kawasaki disease; intravenous immunoglobulins; PIMS/MIS-C; shock syndrome; intensive care unit; SARS-CoV-2-related inflammatory multisystem syndrome; Intravenous Immunoglobulins; Shock syndrome; Intensive Care Unit; SARS-CoV-2–related inflammatory multisystem syndrome; Systemic vasculitis; Kawasaki disease, systemic vasculitis, intensive care unit, shock syndrome, intravenous immunoglobulins, PIMS/MIS-C, SARS-CoV-2-related inflammatory multisystem syndrome, COVID-19
• ISSN: 1462-0324; 1462-0332; 1460-2172
• DOI: 10.1093/rheumatology/keab026

Objective To better define the clinical distinctions between the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related paediatric inflammatory multisystem syndrome (PIMS) and Kawasaki disease (KD). Methods We compared three groups of patients: group 1, cases from our national historic KD database (KD-HIS), before the SARS-CoV-2 pandemic; group 2, patients with KD admitted to an intensive care unit (KD-ICU) from both our original cohort and the literature, before the SARS-CoV-2 pandemic; and group 3, patients with PIMS from the literature. Results KD-HIS included 425 patients [male:female ratio 1.3, mean age 2.8 years (s.d. 2.4)], KD-ICU 176 patients [male:female ratio 1.3, mean age 3.5 years (s.d. 3.1)] and PIMS 404 patients [male:female ratio 1.4, mean age 8.8 years (s.d. 3.7)]. As compared with KD-HIS patients, KD-ICU and PIMS patients had a higher proportion of cardiac failure, digestive and neurological signs. KD-ICU and PIMS patients also had a lower frequency of typical KD-mucocutaneous signs, lower platelet count, higher CRP and lower sodium level. As compared with KD-HIS and KD-ICU patients, PIMS patients were older and more frequently had myocarditis; they also had fewer coronary abnormalities and lower sodium levels. Unresponsiveness to IVIG was more frequent in KD-ICU than KD-HIS and PIMS patients. Conclusion On clinical grounds, KD-HIS, KD-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.