Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 mimicking Kawasaki disease (Kawa-COVID-19): a multicentre cohort

Marie Pouletty, Charlotte Borocco, Naim Ouldali, Marion Caseris, Romain Basmaci, Noémie Lachaume, Philippe Bensaid, Samia Pichard, Hanane Kouider, Guillaume Morelle, Irina Craiu, Corinne Pondarre, Anna Deho, Arielle Maroni, Mehdi Oualha, Zahir Amoura, Julien Haroche, Juliette Chommeloux, Fanny Bajolle, Constance Beyler, Stéphane Bonacorsi, Guislaine Carcelain, Isabelle Koné-Paut, Brigitte Bader-Meunier, Albert Faye, Ulrich Meinzer, Caroline Galeotti, Isabelle Melki, Marie Pouletty, Charlotte Borocco, Naim Ouldali, Marion Caseris, Romain Basmaci, Noémie Lachaume, Philippe Bensaid, Samia Pichard, Hanane Kouider, Guillaume Morelle, Irina Craiu, Corinne Pondarre, Anna Deho, Arielle Maroni, Mehdi Oualha, Zahir Amoura, Julien Haroche, Juliette Chommeloux, Fanny Bajolle, Constance Beyler, Stéphane Bonacorsi, Guislaine Carcelain, Isabelle Koné-Paut, Brigitte Bader-Meunier, Albert Faye, Ulrich Meinzer, Caroline Galeotti, Isabelle Melki

Abstract

Background: Current data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities.

Methods: Multicentre compilation of patients with KD in Paris region since April 2020, associated with the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ('Kawa-COVID-19'). A historical cohort of 'classical' KD served as a comparator.

Results: Sixteen patients were included (sex ratio=1, median age 10 years IQR (4·7 to 12.5)). SARS-CoV-2 was detected in 12 cases (69%), while a further three cases had documented recent contact with a quantitative PCR-positive individual (19%). Cardiac involvement included myocarditis in 44% (n=7). Factors prognostic for the development of severe disease (ie, requiring intensive care, n=7) were age over 5 years and ferritinaemia >1400 µg/L. Only five patients (31%) were successfully treated with a single intravenous immunoglobulin (IVIg) infusion, while 10 patients (62%) required a second line of treatment. The Kawa-COVID-19 cohort differed from a comparator group of 'classical' KD by older age at onset 10 vs 2 years (p<0.0001), lower platelet count (188 vs 383 G/L (p<0.0001)), a higher rate of myocarditis 7/16 vs 3/220 (p=0.0001) and resistance to first IVIg treatment 10/16 vs 45/220 (p=0.004).

Conclusion: Kawa-COVID-19 likely represents a new systemic inflammatory syndrome temporally associated with SARS-CoV-2 infection in children. Further prospective international studies are necessary to confirm these findings and better understand the pathophysiology of Kawa-COVID-19. Trial registration number NCT02377245.

Keywords: cytokines; inflammation; outcome and process assessment, health care.

Conflict of interest statement

Competing interests: None declared.

© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Figures

Figure 1
Figure 1
Clinical features of Kawa-COVID-19 patients. (1A) Chest CT scan (lung window) of a 12-year-old girl with Kawasaki disease and SARS-CoV-2 infection showing diffuse peripheral ground-glass opacities in both lungs. (1B-1E) Mucocutaneous lesions in a 4.5-year-old boy with Kawasaki disease and SARS-CoV-2 infection: Non-purulent conjunctivitis (1B), maculo-papular rash (1C), dry cracked lips (1D) and orchitis (1E).
Figure 2
Figure 2
Main clinical features of Kawa-COVID-19 from the Paris region cohort, n=16 patients. In red: higher frequencies than classical Kawasaki disease.

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