Management of Multisystem Inflammatory Syndrome in Children Associated With COVID-19: A Survey From the International Kawasaki Disease Registry

Abstract

Background Since April 2020, there have been numerous reports of children presenting with systemic inflammation, often in critical condition, and with evidence of recent infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This condition, since defined as the multisystem inflammatory syndrome in children (MIS-C), is assumed to be a delayed immune response to COVID-19, and there are frequently cardiac manifestations of ventricular dysfunction and/or coronary artery dilation. Methods We surveyed the inpatient MIS-C management approaches of the members of the International Kawasaki Disease Registry across 38 institutions and 11 countries. Results Among the respondents, 56% reported using immunomodulatory treatment for all MIS-C patients, regardless of presentation. Every respondent reported use of intravenous immunoglobulin (IVIG), including 53% administering IVIG in all patients. Steroids were most often used for patients with severe clinical presentation or lack of response to IVIG, and only a minority used steroids in all patients (14%). ASA was frequently used among respondents (91%), including anti-inflammatory and/or anti-platelet dosing. Respondents reported use of prophylactic anticoagulation, especially in patients at higher risk for venous thromboembolism, and therapeutic anticoagulation, particularly for patients with giant coronary artery aneurysms. Conclusions There is variation in management of MIS-C patients with suboptimal evidence to assess superiority of the various treatments; evidence-based gaps in knowledge should be addressed through worldwide collaboration to optimize treatment strategies.

The “Golden Hours” Algorithm For the Management of the Multisystem Inflammatory Syndrome in Children (MIS-C)

“…Although most of the respondents of the International Kawasaki Disease Registry (IKDR) across 38 institutions and 11 countries reported a preference of IL-1Ra (58%) and TNF-a inhibitor (28%) 25 use as compared to IL-6Ra (8%), in our case series we resorted to the use of IL-6Ra in one patient due to the availability of the drug as well as the markedly elevated level of IL-6.…”

Regression of Coronary Aneurysms With Intravenous Immunoglobulins and Steroids for COVID-19 Adult Multisystem Inflammatory Syndrome

“…Although treatment with IVIG is effective in the acute phase of Kawasaki disease, its effectiveness in preventing the development of aneurysms continues to be debated ( 8 ). A recent report from the International Kawasaki Disease Registry states that IVIG is the most widely used treatment in the context of PIMS-TS in combination with steroids in worrying situations ( 9 ). Due to the severity of the clinical picture and the lack of evidenced-based data regarding the management of this condition, both treatments were used simultaneously in our patient, with a good clinical outcome.…”

Síndrome Inflamatória Multissistêmica Pediátrica (SIM-P) temporariamente associada à COVID-19: um levantamento das características clínicas e epidemiológicas

“…Durante a infecção das células dendríticas ou macrófagos pelo SARS-CoV-2 ocorre uma indução de reduzidos níveis de citocinas antivirais e elevação da síntese de citocinas inflamatórias, como o fator de necrose tumoral [TNF], interleucina [IL] -1, IL-6, IL-10 e interferon-y, que levam a expressão do fator tecidual, liberação de quininas inflamatórias e redução do número total de linfócitos, afetando a imunidade inata e adquirida. Assim, verifica-se uma desregulação excessiva da resposta imune, promovendo o desenvolvimento de hiperinflamação por meio da liberação desses mediadores (Jiang et al, 2020;Elias et al,2020;Alunno, Carubbi & Rodríguez-Carrio, 2020).…”