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Multisystem inflammatory syndrome in children associated with COVID-19 may share overlapping clinical features with Kawasaki Disease unrelated to the pandemic, according to guidelines released by the American College of Rheumatology.
The ACR announced the “Clinical Guidance for Pediatric Patients with Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with SARS-CoV-2 and Hyperinflammation in COVID-19” on June 18, alongside another set of COVID-19 guidance for pediatric patients with rheumatic disease. Both documents are pending journal peer review.
“The ACR has been receiving calls from primary care providers and emergency medicine physicians who are understandably concerned about MIS-C given that it can progress to shock and serious cardiac consequences for patients,” Jay Mehta, MD, MS, of the Children’s Hospital of Philadelphia, who oversaw the task force that drafted the guidelines, told Healio Rheumatology. “Prior to April 2020, the condition had never been reported and there was considerable uncertainty about how to diagnose and treat the illness.”
“Likewise, there are parents who have been understandably worried when their children develop a fever and rash that seem to mimic MIS-C symptoms,” he added. “In most instances, the fever and rash do not cause shock and are fairly common symptoms of many viral illnesses. Although the vast majority of children with fever will have a condition other than MIS-C, we felt it was important to provide a diagnostic approach and treatment recommendations for patients that showed symptoms, confirmed by a diagnostic evaluation, consistent with MIS-C.”
To provide guidance on the management of inflammatory syndromes in children with recent or concurrent infections with severe COVID-19, the ACR assembled a task force of nine pediatric rheumatologists, two adult rheumatologists, two pediatric cardiologists, two pediatric infectious disease specialists and one pediatric critical care physician. The panel’s first meeting, on May 22, saw members form four workgroups to address clinical questions related to MIS-C and hyperinflammation in COVID-19. Each workgroup generated preliminary statements supported by evidence and shared with the entire task force.
Members then conducted two rounds of anonymous voting, as well as two webinars in which they discussed the results to achieve consensus. They also used a nine-point scale to determine the appropriateness of each statement, with consensus rated as either low, moderate or high based on dispersion of the votes along the numeric scale. Approved guidance statements had to be classified as either moderate or high on the consensus scale.
According to ACR, the guidance offers direction on the diagnostic evaluation of MIS-C, compares MIS-C to Kawasaki Disease, and provides general recommendations for cardiac management, immunotherapy treatment and anti-blood clotting therapies in MIS-C. In all, the document included 41 recommendations. They include:
MIS-C and Kawasaki Disease unrelated to COVID-19 infections may share overlapping features, including conjunctival injection, red or cracked lips, “strawberry tongue,” rash, swollen or erythematous hands and feet, and cervical lymphadenopathy;
For cardiac management, EKGs should be performed every 48 hours, at a minimum, in patients with MIS-C patients who are hospitalized as well as during follow-up;
For anti-blood clotting therapy, daily, low dose aspirin not exceeding 81 mg per day is recommended in patients with MIS-C and Kawasaki Disease-like features, or those with a platelet count of 450,000 per µL or greater, until the platelet count is normal and there is confirmed normal coronary arteries at 4 weeks or longer following diagnosis;
Aspirin should be avoided in patients with a platelet count of less than 80,000 per µL;
Children with severe respiratory symptoms due to COVID-19 with acute respiratory distress syndrome, shock/cardiac dysfunction, elevated lactate dehydrogenase enzyme, D-dimer, IL-6, IL-2R or ferritin, along with depressed lymphocyte count, albumin or platelet count, should be considered for immunotherapy; and
Glucocorticoids may be considered for immunomodulatory therapy among patients with COVID-19 and hyperinflammation.
“This document serves as a reminder that even though there is heightened concern about MIS-C, it remains quite a rare condition,” Mehta said. “While these documents should not replace the good, clinical judgement of physicians, in a time of high uncertainty, like now, they can provide a framework for managing and treating children with a rheumatic disease or children suspected of having MIS-C. As always, any decisions about management should be made in conjunction with the family.”