Abstract
Background: Following the spread of the coronavirus disease 2019 (COVID-19) pandemic a new disease entity emerged, defined as Pediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS), or Multisystem Inflammatory Syndrome in Children (MIS-C). In the absence of trials, evidence for treatment remains scarce.Purpose: To develop best practice recommendations for the diagnosis and treatment of children with PIMS-TS in Switzerland. It is acknowledged that the field is changing rapidly, and regular revisions in the coming months are pre-planned as evidence is increasing.Methods: Consensus guidelines for best practice were established by a multidisciplinary group of Swiss pediatric clinicians with expertise in intensive care, immunology/rheumatology, infectious diseases, hematology, and cardiology. Subsequent to literature review, four working groups established draft recommendations which were subsequently adapted in a modified Delphi process. Recommendations had to reach >80% agreement for acceptance.Results: The group achieved agreement on 26 recommendations, which specify diagnostic approaches and interventions across anti-inflammatory, anti-infectious, and support therapies, and follow-up for children with suspected PIMS-TS. A management algorithm was derived to guide treatment depending on the phenotype of presentation, categorized into PIMS-TS with (a) shock, (b) Kawasaki-disease like, and (c) undifferentiated inflammatory presentation.Conclusion: Available literature on PIMS-TS is limited to retrospective or prospective observational studies. Informed by these cohort studies and indirect evidence from other inflammatory conditions in children and adults, as well as guidelines from international health authorities, the Swiss PIMS-TS recommendations represent best practice guidelines based on currently available knowledge to standardize treatment of children with suspected PIMS-TS. Given the absence of high-grade evidence, regular updates of the recommendations will be warranted, and participation of patients in trials should be encouraged.
Highlights
Subsequent to the first wave of the coronavirus disease 2019 (COVID-19) pandemic [1], clusters of children presenting with unusual multisystem inflammatory conditions emerged [2, 3]
The thereby developed guideline was piloted during 4 weeks in January 2021, subsequent to which an update and re-voting occurred on items which were agreed to be changed in the panel
In patients with PIMS-TS refractory to initial treatment with intravenous immunoglobulin (IVIG) and steroids, and after exclusion of alternative causes by the multidisciplinary team, we suggest consideration for anakinra
Summary
Subsequent to the first wave of the coronavirus disease 2019 (COVID-19) pandemic [1], clusters of children presenting with unusual multisystem inflammatory conditions emerged [2, 3]. The clinical syndrome showed some heterogeneity with patients presenting either akin to toxic shock, Kawasaki-disease like or with undifferentiated inflammatory characteristics, in addition to evidence of current or past COVID-19 infection in the majority of cases [4, 5]. This new disease entity has been called Pediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS) as per the Royal College of Pediatrics and Child Health case definition (RCPCH) [6] and Multisystem Inflammatory Syndrome associated with COVID19 (MIS-C) as per the Center for Disease Control (CDC) [7] and the WHO (World Health Organisation) [8]. It is acknowledged that the field is changing rapidly, and regular revisions in the coming months are pre-planned as evidence is increasing
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