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Best Practice Recommendations for the Diagnosis and Management of Children With Pediatric Inflammatory Multisystem
Luregn J Schlapbach1,2, Maya C Andre3,4, Serge Grazioli5
1Pediatric and Neonatal Intensive Care Unit, Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.
Insights
New guidelines offer best practices for diagnosing and treating Pediatric Inflammatory Multisystem Syndrome (PIMS-TS) in children. These recommendations address anti-inflammatory, anti-infectious, and supportive therapies, aiming to standardize care for PIMS-TS in Switzerland.
Area of Science:
- Pediatric critical care medicine
- Pediatric rheumatology
- Pediatric infectious diseases
Background:
- The COVID-19 pandemic led to the emergence of Pediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS), also known as Multisystem Inflammatory Syndrome in Children (MIS-C).
- Limited evidence exists for effective PIMS-TS treatments due to the scarcity of clinical trials.
Purpose of the Study:
- To establish best practice recommendations for the diagnosis and treatment of PIMS-TS in children within Switzerland.
- To provide a framework for managing PIMS-TS, acknowledging the rapidly evolving nature of the condition and planned future revisions.
Main Methods:
- A multidisciplinary group of Swiss pediatric specialists collaborated to develop consensus guidelines.
- Recommendations were drafted by four working groups after a literature review and refined using a modified Delphi process, requiring over 80% agreement.
Main Results:
- Twenty-six recommendations were agreed upon, covering diagnostic approaches, interventions (anti-inflammatory, anti-infectious, supportive), and follow-up care for PIMS-TS.
- A management algorithm was developed to guide treatment based on clinical presentation, including shock, Kawasaki-disease-like symptoms, or undifferentiated inflammation.
Conclusions:
- Current PIMS-TS literature primarily consists of observational studies, necessitating the development of best practice guidelines.
- These Swiss PIMS-TS recommendations aim to standardize care based on available evidence and international guidelines, with updates planned as new data emerges.
- Encouraging patient participation in clinical trials is crucial for advancing high-grade evidence in PIMS-TS management.
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.
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