American College of Rheumatology Clinical Guidance for Pediatric Patients with Multisystem Inflammatory Syndrome in Children (MIS‐C) Associated with SARS‐CoV‐2 and Hyperinflammation in COVID‐19. Version 1

Henderson, Lauren A.; Canna, Scott W.; Friedman, Kevin G.; Gorelik, Mark; Lapidus, Sivia K.; Bassiri, Hamid; Behrens, Edward M.; Ferris, Anne; Kernan, Kate F.; Schulert, Grant S.; Seo, Philip; F. Son, Mary Beth; Tremoulet, Adriana H.; Yeung, Rae S.M.; Mudano, Amy S.; Turner, Amy S.; Karp, David R.; Mehta, Jay J.

OBJECTIVE: To provide guidance on the management of Multisystem Inflammatory Syndrome in Children (MIS‐C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests late in the course of SARS‐CoV‐2 infection. The Task Force also provided recommendations for children with hyperinflammation during COVID‐19, the acute, infectious phase of SARS‐CoV‐2 infection. METHODS: The Task Force was composed of 9 pediatric rheumatologists, 2 adult rheumatologists, 2 pediatric cardiologists, 2 pediatric infectious disease specialists, and 1 pediatric critical care physician. Preliminary statements addressing clinical questions related to MIS‐C and hyperinflammation in COVID‐19 were developed based on evidence reports. Consensus was built through a modified Delphi process that involved 2 rounds of anonymous voting and 2 webinars. A 9‐point scale was used to determine the appropriateness of each statement (1‐3, inappropriate; 4‐6, uncertain; 7‐9, appropriate), and consensus was rated as low (L), moderate (M), or high (H) based on dispersion of the votes along the numeric scale. Approved guidance statements had to be classified as appropriate with moderate or high levels of consensus, which were pre‐specified prior to voting. RESULTS: A total of 128 statements were approved by the Task Force, which were refined into 40 final guidance statements accompanied by a flow diagram depicting the diagnostic pathway for MIS‐C. CONCLUSION: Our understanding of SARS‐CoV‐2‐related syndromes in the pediatric population continues to evolve. This guidance document reflects currently available evidence coupled with expert opinion but is meant to be modified as additional data become available.