AAP issues guidance on multisystem inflammatory syndrome in children
The AAP has developed guidance on identifying, testing and treating children with multisystem inflammatory syndrome, which has been associated with COVID-19.
In May, the CDC issued a health advisory that provided a case definition of multisystem inflammatory syndrome in children (MIS-C):
- A patient aged younger than 21 years who presents with a fever, has evidence of inflammation and has multisystem organ involvement.
- The patient has no alternative plausible diagnoses.
- The patient is positive for current or recent SARS-CoV-2 infection or has been exposed to COVID-19 within the 4 weeks prior to developing symptoms.
Sandy Hong, MD, the chair of the AAP’s section on rheumatology and co-author of the MIS-C guidelines, said it is important to realize that MIS-C might present differently.
“We want people to be able to recognize [MIS-C], to realize it is rare, but that it comes in multiple flavors,” Hong told Healio. “It comes with a Kawasaki-like syndrome or the toxic shock-like syndrome or the cytokine storm-like syndrome. Actually, a cytokine storm can happen with either presentation. We want people to realize that it is rare, it does exist and it's not going to happen in the country all at the same time.”
Hong said the guidance will be applicable for those in COVID-19 “hot spots” around the country as well as in areas where the hot spots are “quieting down” — research suggests the onset MIS-C can be delayed following the primary infection. She said that because states reopened at different times, hot spots for MIS-C cases will differ around the country.
“I think one of the things we wanted people to realize, and we said it subtly, and not so subtly, is that this is a rare condition that is temporally associated with COVID-19,” Hong said. “So, if you don't have that much COVID-19 in your neighborhood, you shouldn't be looking for it; but if you have a lot in your neighborhood, then you need to start looking for it.”
Hong said it was challenging to write proper guidelines because MIS-C presents differently in each child.
“The one is the child with presentation of a fever without a source” for 3 days or more, she said, “but then there's the other presentation of toxic shock-like syndrome in kids who basically show up with compensated shock. They become very sick, very quickly and need critical care access within hours and so, it was difficult making these guidelines because of the different syndromes that these children are presenting.”
According to the guidance, children who present with symptoms may undergo expanded laboratory testing and cardiac workup that may include the following:
- X-ray and ECG;
- expanded laboratory tests including troponin, pro-B-type natriuretic peptide, triglycerides, creatine kinase, amylase, blood and urine culture, D-dimer, prothrombin time/partial thromboplastin time, international normalized ratio, CRP, ferritin, lactic acid dehydrogenase, comprehensive metabolic panel and fibrinogen, if not already conducted; and
- COVID-19 testing performed with RT-PCR assay and serologic testing in every case.
Hong recommended that any pediatrician or pediatric ID specialist should, in no case, try to treat a child with MIS-C alone.
“I think you shouldn't try to [treat a patient] alone because they'll need a rheumatologist, they'll need a cardiologist, they may or may not need an intensivist, and they probably will need an immunologist and maybe even a hematologist because of the coagulopathies that can occur,” Hong said. “My message for pediatricians who are reading the guidelines — I think it quickly becomes clear that MIS-C patients require a team.”