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October 06, 2020
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‘We don’t know why this happens’: When to suspect MIS-C in a patient

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Multisystem inflammatory syndrome in children is rare, occurring in one or two out of every 1,000 reported pediatric patients with COVID-19, Sandy Hong, MD, FAAP, noted in a presentation during the AAP National Conference & Exhibition.

It occurs mostly in centers with large populations, Hong said. She said physicians should consider multisystem inflammatory syndrome in children (MIS-C) as they evaluate patients for other illnesses if there is COVID-19 activity in the area.

Sandy Hong

“We don’t know why this happens,” Hong told Healio. “We think it is due to an abnormal and intense immune response. We do not know what part of the virus is responsible and which specific parts of the immune system are triggering the abnormal response.”

Hong, who is chair of the AAP’s section on rheumatology and a co-author of AAP’s MIS-C guidelines, noted that MIS-C appears to be a delayed reaction caused by COVID-19.

According to the CDC, as of Oct. 1, there have been 1,027 confirmed cases of MIS-C in 44 states, New York City and Washington, D.C., and 20 deaths from the condition.

Hong reminded attendees about the CDC case definition for MIS-C:

  • A patient aged younger than 21 years, presenting with fever, laboratory evidence of inflammation and evidence of clinically severe illness requiring hospitalization, with multisystem organ involvement; and
  • The patient has no alternative plausible diagnosis; and
  • The patient is positive for current or recent SARS-CoV-2 infection by RT-PCR, serology or antigen test; or has been exposed to COVID-19 within 4 weeks before developing symptoms.

According to the CDC, there have not been studies comparing the clinical efficacy of treatments for MIS-C, which “have consisted primarily of supportive care and directed care against the underlying inflammatory process.”

The CDC does list several supportive measures for patients with MIS-C: fluid resuscitation, inotropic support, respiratory support and, in rare cases, extracorporeal membranous oxygenation.

“Anti-inflammatory measures have included the frequent use of IVIG and steroids,” the CDC notes. “The use of other anti-inflammatory medications and the use of anti-coagulation treatments have been variable. Aspirin has commonly been used due to concerns for coronary artery involvement, and antibiotics are routinely used to treat potential sepsis while awaiting bacterial cultures. Thrombotic prophylaxis is often used given the hypercoagulable state typically associated with MIS-C.”

“MIS-C, similar to Kawasaki disease, is a postinfectious immune response to an antigen,” Hong said. “Treatment for MIS-C will differ from classic Kawasaki disease due to the fact that we know the SARS-CoV-2 virus affects the lungs, heart, brain and causes coagulopathy in some. This reality will affect and influence which medications and specialist will be needed. Treatment of MIS-C requires a team approach.”

This month, researchers reported in MMWR that a condition similar to MIS-C has been identified in adults, which the researchers called MIS-A.

“We know there have been rare adult cases of Kawasaki disease. So I suspect that there will be rare cases of MIS-C in adults, too,” Hong said.