Background

In late April 2020, reports of cases of myocarditis with cardiogenic shock in children with a recent COVID-19 infection led Santé publique France and pediatric medical societies* to implement active surveillance of these atypical clinical presentations.

These syndromes have been described in many countries and are classified under the term “pediatric multisystem inflammatory syndrome” (PIMS: Pediatric inflammatory multisystem syndrome, or MIS-C: Multisystem inflammatory syndrome in children).

Definition of a PIMS or MIS-C case

The definition of a case of PIMS or MIS-C is as follows (WHO):

Children and adolescents aged 0 to 19 years with a fever lasting ≥3 days:

• AND presenting with at least two of the following signs:

  • a) rash or bilateral non-purulent conjunctivitis or signs of mucocutaneous inflammation (mouth, hands, or feet);

  • b) hypotension or shock;

  • c) signs of myocardial dysfunction, pericarditis, valvulitis, or coronary abnormalities (abnormalities on echocardiography or elevated troponin/NT-proBNP levels);

  • d) findings suggestive of coagulopathy (abnormal prothrombin time, activated partial thromboplastin time, elevated D-dimer levels);

  • e) acute gastrointestinal symptoms (diarrhea, vomiting, or abdominal pain);

• AND elevated markers of inflammation such as ESR, C-reactive protein, or procalcitonin

• AND no other obvious microbial cause of inflammation, such as bacterial sepsis or staphylococcal or streptococcal shock syndromes

• AND evidence of COVID-19 (via RT-PCR, antigen test, or positive serology) or probable contact with patients with COVID-19.

Note: This syndrome should be considered in children presenting with features of typical or atypical Kawasaki disease or toxic shock syndrome.

The French National Authority for Health has developed rapid guidelines for healthcare professionals, particularly general practitioners and pediatricians, on the identification of and management of children presenting with this syndrome.

Objectives of the surveillance

The initial objective of this surveillance was to confirm the warning signal regarding the emergence of this new “Kawasaki-like” syndrome linked to COVID-19, and then to identify cases. The identification of these cases should subsequently enable the launch of specific studies initiated by pediatric professional societies.

Methods

In order to best identify these atypical clinical presentations that appear to correspond to a post-COVID-19 post-infectious syndrome, since April 30, 2020, hospital pediatricians have been asked, through pediatric medical societies, to report to Santé publique France every new case of atypical pediatric multisystem inflammatory syndrome, possibly linked to COVID-19, as well as retrospectively since March 1, 2020.

To enable pediatricians to report these cases online, Santé publique France has developed a dedicated platform.

The detailed methodology and initial results of this surveillance in France were published in June 2020.

The HAS has developed rapid guidance for healthcare professionals, particularly general practitioners and pediatricians, on identifying and managing children presenting with this syndrome.