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Multisystem inflammatory syndrome

Key sources
The following summarized guidelines for the evaluation and management of multisystem inflammatory syndrome are prepared by our editorial team based on guidelines from the World Health Organization (WHO 2023), the American College of Rheumatology (ACR 2022), and the Center for Disease Control (CDC 2021; 2020).
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Guidelines

1.Screening and diagnosis

Epidemiology
Recognize that the vast majority of pediatric patients with COVID-19 present with mild symptoms and have excellent outcomes, and the MIS-C remains a rare complication of SARS-CoV-2 infections.
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Recognize that MIS-C is temporally associated with SARS-CoV-2 infections. Use the prevalence of the virus in a given geographic location, which may change over time, to inform management decisions.
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  • Definitions

  • Differential diagnosis

2.Diagnostic investigations

Initial evaluation: as per ACR 2022 guidelines, obtain diagnostic evaluation for MIS-C in patients under investigation without life-threatening manifestations before initiating immunomodulatory treatment.
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  • Diagnostic imaging

  • Evaluation for other causes

3.Diagnostic procedures

Lumbar puncture: consider performing lumbar puncture in patients under investigation for MIS-C.
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4.Medical management

General principles: consider offering close monitoring only without immunomodulatory therapy in some patients with mild symptoms, after evaluation by specialists with expertise in MIS-C.
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  • Setting of care

  • IVIG

  • Corticosteroids

  • Biologics

  • Antiplatelet and anticoagulant therapy

5.Specific circumstances

Patients with hyperinflammation syndrome: recognize that pediatric patients, particularly infants, with medical complexity including T1DM, complex congenital heart disease, neurologic conditions, obesity, or asthma and patients receiving immunosuppressive medications may be at higher risk for severe COVID-19. Recognize that racial and ethnic minorities may also be at higher risk.
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6.Follow-up and surveillance

Serial cardiac monitoring: monitor BNP and/or troponin T levels until normalization in patients with MIS-C with abnormalities of these laboratory parameters at diagnosis.
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