Abstract

Backgrounds:

The multisystem inflammatory syndrome in children (MIS-C) is a rare but severe hyperinflammatory condition that may occur following SARS-CoV-2 infection.

Methods

This is a retrospective, descriptive study of patients hospitalized with MIS-C in 10 tertiary care centers in Greece until December 31st, 2021. Demographics, clinical and laboratory characteristics, treatment and outcome are described.

Results:

119 patients (78 males, median age 8.4 years, IQR 4.5-13.5) were included and 109/119 met the criteria of the WHO case definition of MIS-C while 105/119 (88.2%) had serological evidence of SARS-CoV-2 infection. Of the 119 patients, fever was present in 99.2%, gastrointestinal symptoms in 79%, mucocutaneous involvement in 64% and respiratory symptoms in 32%. Forty-five patients (38%) developed myocarditis, 26 (22%) pericarditis and 7 (6%) coronary aneurysms. In the above cases 8/45 (17.7%), 1/26 (3.8%) and 3/7 (42.8%) respectively, cardiac complications had not fully resolved at discharge. Underlying comorbidities were reported in 22/119 (18.5%) patients. Median CRP, ferritin and WBC values were 134mg/L (IQR, 30.9-228), 453ng/ml (IQR, 275.5-987.3) and 14.080/mm³ (IQR, 9495-20305), respectively. The majority of patients had elevated troponin (82/119, 68.9%) and/or pro-BNP (53/119, 44.5%). Intravenous immunoglobulin and/or corticosteroids were used in 101/119 (85%) whereas anti-IL1 treatment was added in 9/119 (7.6%). 25 patients (21%) were admitted to the ICU, 13 (10.9%) developed shock and one (0.8%) patient required ECMO. Mortality rate was 0.9%. The incidence of MIS-C was estimated at 0.64/1000 SARS-CoV-2 infections.

Conclusions/Learning Points:

MIS-C is a novel, infrequent but serious disease entity. Most common cardiac manifestations included myocarditis and pericarditis, which resolved in the majority of our patients. Immunomodulatory therapy was shown to be effective however residual cardiac involvement remains an issue. Further research is required to elucidate the pathogenesis, risk factors and optimal management.

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