COVID-19 Update: Multisystem Inflammatory Syndrome in Children (MIS-C)

by: Salwa Gendi, MD, FCP and Michelle Cretella, MD, FCP

Since late April, a systemic immune-mediated inflammatory disease, similar to Kawasaki disease and Toxic Shock Syndrome, associated with SARS-CoV-2 infection, has been observed to occur in some children. As of May 22, 2020, 5 European countries and nearly half of US states have reported this syndrome, with the state of New York logging 161 cases including 3 deaths.1


The CDC recommends reporting MIS-C

Health professionals should report any patient who meets the case definition below to local, state, and territorial health departments.


Case Definition for MIS-C:

A patient under 21 years of age who meet all of the following criteria:

  • Fever greater than or equal to 100 degrees Fahrenheit for at least 24 hours, or report of subjective fever of at least 24 hours.

  • Laboratory evidence of inflammation (including but not limited to elevated, CRP, ESR, fibrinogen, pro-calcitonin, d-dimer, ferritin, LDH, IL-6, neutrophils, and/or reduced lymphocytes and low albumin).

  • Evidence of clinically severe multisystem illness requiring hospitalization that involves at least 2 organ systems.

  • No alternative diagnosis.

  • Positive for current or recent SARS-CoV-2 infection by RT-PCR, serology, or antigen test; or COVID-19 exposure within 4 weeks prior to the onset of symptoms.

Additional notes:

  • Children may meet full or partial criteria for Kawasaki disease

  • Consider MIS-C in any pediatric death with evidence of SARS-CoV-2 infection

Treatment:

  • No proven treatment established

  • Good results have been reported following Kawasaki Disease protocols to reduce swelling, fight infection and protect vital organs by prescribing:

    1. Corticosteroids

    2. IVIG

    3. High dose Aspirin 

    4. Other meds as indicated (eg: Anakinra)

Clinical Presentation of Kawasaki Disease3

Kawasaki disease signs and symptoms usually appear in three phases.

1st phase

Signs and symptoms of the first phase may include:

  • A fever that is often is higher than 102.2 F (39 C) and lasts five or more days4

  • Extremely red eyes without a thick discharge

  • A rash on the main part of the body and in the genital area

  • Red, dry, cracked lips and an extremely red, swollen tongue

  • Swollen, red skin on the palms of the hands and the soles of the feet

  • Swollen lymph nodes in the neck and perhaps elsewhere

  • Irritability

2nd phase

In the second phase of the disease, your child may develop:

  • Peeling of the skin on the hands and feet, especially the tips of the fingers and toes, often in large sheets

  • Joint pain

  • Diarrhea

  • Vomiting

  • Abdominal pain

 3rd phase

  • In the third phase of the disease, signs and symptoms slowly go away unless complications develop. It may be as long as eight weeks before energy levels seem normal again.

Salwa Gendi, MD, FCP is Assistant Professor of Pediatrics at West Virginia University School of Medicine and a pediatric cardiologist.

Michelle Cretella, MD, FCP is Executive Director of the American College of Pediatricians

References

1  Russo  M. 161 Severe Child COVID Syndrome Cases Now Being Investigated in NY: Docs Fear Undercount. NBC News 4. Updated May 22, 2020. Available at: .https://www.nbcnewyork.com/news/local/new-york-investigating-157-cases-of-pediatric-covid-illness-mis-c-cuomo-says/2426986/ Accessed May 26, 2020.

2.  How is Multisystem Inflammatory Syndrome in Children Treated? Nicklaus Children’s Hospital Website updated May 22, 2020. Available at: https://www.nicklauschildrens.org/conditions-we-treat/multisystem-inflammatory-syndrome-(mis-c) Accessed May 26, 2020.

3. Kawasaki Disease: an overview by the Mayo Clinic. Available from: https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598 Accessed May 26, 2020.

4. Kawasaki Disease: Case Definition by the CDC. Available from: https://www.cdc.gov/kawasaki/case-definition.html Accessed May 28, 2020