key: cord-0816360-kwtpmsk0 authors: Shaykh, Ramzi; Leftin, Shoshana; Suh, Sanghee; Spencer, Robert; Gorelik, Mark; Wilson, Patrick T.; Diamond, Rebekah title: Reversible Coronary Artery Aneurysm With Delayed Anti-inflammatory Therapy in Multisystem Inflammatory Syndrome in Children date: 2021-02-24 journal: JACC Case Rep DOI: 10.1016/j.jaccas.2020.11.045 sha: cc31081aaba6ddf747677311b369b72f9be94c1c doc_id: 816360 cord_uid: kwtpmsk0 A 4-year-old boy with multisystem inflammatory syndrome in children before widespread recognition of this disease developed complications, including coronary artery aneurysm, without anti-inflammatory treatment. With delayed treatment, all sequelae resolved. This case demonstrates a natural history supporting the role of anti-inflammatory treatment even with delayed or equivocal diagnosis. (Level of Difficulty: Intermediate.) S evere acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) has created urgent challenges for the medical community. Although published scientific reports have focused on the adult population, the emergence of multisystem inflammatory syndrome in children (MIS-C) has brought focus to how SARS-CoV-2 can affect children (1) (2) (3) (4) . Because of the severity of MIS-C, which may present with lifethreatening shock, myocarditis, and coagulopathy, the pediatric community has quickly developed management guidelines. However, as we await further studies, treatment remains founded on limited evidence and expert consensus (5, 6) . Current guidelines for MIS-C emphasize its similarities to Kawasaki disease (KD), a rare childhood vasculitis associated with coronary artery aneurysm formation. Typical KD is marked by prolonged fever and diffuse mucocutaneous inflammation, whereas incomplete KD presents with fever and derangements in inflammatory markers. We describe a patient with MIS-C who presented before widespread recognition of this disease, leading to delayed diagnosis. To our knowledge, this is the only described case of untreated MIS-C with sequelae, including coronary To understand the potential natural history of untreated MIS-C, including coronary artery aneurysm formation, and compare it to that of KD. To review the importance of antiinflammatory treatment of MIS-C in aneurysm prevention and treatment and to evaluate the role of treatment even with delayed or equivocal diagnosis. There was no significant medical history. Presumed diagnosis was viral sepsis due to acute SARS-CoV-2 infection. Bacterial pneumonia and viral myocarditis were also high on the differential diagnosis. (Table 1 ) showed a One notable divergence between this case and KDrelated coronary artery aneurysms is the rapid return to normal luminal dimension less than 1 week after initiation of treatment. Current treatment pathways for KD recommend anti-inflammatory management until at least 10 days after presentation for maximal efficacy in preventing coronary artery formation (8, 9) . In our case, treatment was delayed well beyond the typical windows for KD yet resulted in even faster aneurysm resolution than is typically seen in KD, where comparable aneurysms demonstrate improvement 6 to 18 months after treatment (8) . CDC Health Alert Network. Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease Acute heart failure in multisystem inflammatory syndrome in children in the context of global SARS-CoV-2 pandemic Clinical characteristics of 58 children with a pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 Multisystem inflammatory syndrome in children in New York State COVID-19 and multisystem inflammatory syndrome in children and adolescents CDC COVID-19 Response Team. Coronavirus disease 2019 in children-United States Delayed diagnosis of Kawasaki syndrome: an analysis of the problem Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association Prevalence of Kawasaki disease in young adults with suspected myocardial ischemia Multisystem inflammatory syndrome related to COVID-19 in previously healthy children and adolescents in New York City ACKNOWLEDGMENTS The authors thank the patient and his family for allowing us to share their story.And they thank the Pediatric Emergency Medicine, Pediatric Intensive Care Unit, and Progressive Care Unit medical, nursing, consultant, and support teams for their excellent care. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.