Multisystem inflammatory syndrome with features of Atypical Kawasaki disease during COVID-19 pandemic: Report of a case from India

Abstract

There is a global concern of increasing number of children presenting with inflammatory syndrome with clinical features simulating Kawasaki disease, during ongoing COVID-19 pandemic. We report a very similar case of 5-year-old boy from a COVID-19 hotspot area in Kerala state of India who presented in late April 2020 with acute febrile illness with abdominal pain and loose stools followed by shock. On examination, child had bulbar conjunctivitis and extremity edema. Initial investigations showed high inflammatory parameters, elevated serum creatinine and liver enzymes. Echocardiography showed moderate LV dysfunction and normal coronaries. Cardiac enzymes were also elevated, suggesting myocarditis. He was treated with inotropic support, respiratory support with High Flow Nasal Cannula, IV Immunoglobulins, aspirin, steroids and diuretics. RT PCR for SARS-CoV-2 was negative twice. His clinical condition improved rapidly, was afebrile from day 2, inflammatory parameters decreased, left ventricular function improved and was discharged after 6 days of hospital stay.

Mapping Systemic Inflammation and Antibody Responses in Multisystem Inflammatory Syndrome in Children (MIS-C)

“…The extent to which genetics impacts the development of MIS-C is currently unclear. Certainly, Black or Hispanic ethnicity appears to be a major risk factor, as observed in this study and others ( Cheung et al, 2020 ; Jones et al, 2020 ; Klocperk et al, 2020 ; Rauf et al ; Riphagen et al, 2020 ; Toubiana et al, 2020 ; Verdoni et al, 2020 ; Whittaker et al, 2020 ). This enrichment diverges from KD, in which the incidence is significantly higher in children of Asian ancestry.…”

“…While adults have suffered the highest rates of morbidity and mortality of COVID-19, children were thought to be spared ( Dong et al, 2020 ; Ludvigsson, 2020 ). Recently, however, cases of hyperinflammatory shock in children have been reported in regions with receding SARS-CoV-2 epidemics ( Cheung et al, 2020 ; Jones et al, 2020 ; Klocperk et al, 2020 ; Rauf et al ; Riphagen et al, 2020 ; Toubiana et al, 2020 ; Verdoni et al, 2020 ; Whittaker et al, 2020 ).…”

“…Initially, the syndrome was considered an atypical form of Kawasaki disease (KD), an acute systemic vasculitis in young children, given the presence of fever, rash, conjunctivitis, mucocutaneous involvement and cardiac complications ( Kawasaki, 1967 ; Kawasaki et al, 1974 ). However, it has become evident that shock, gastrointestinal symptoms, and coagulopathy, which are rarely seen in classic KD, are prominent features of this unique syndrome ( Cheung et al, 2020 ; Jones et al, 2020 ; Klocperk et al, 2020 ; Rauf et al ; Riphagen et al, 2020 ; Toubiana et al, 2020 ; Verdoni et al, 2020 ; Whittaker et al, 2020 ). Furthermore, Black and older children appear disproportionately affected, in contrast to the association of young children of Asian descent with KD ( Holman et al, 2010 ; Nakamura et al, 2010 ).…”

“…The concentration of this disease to regions of high local SARS-CoV-2 transmission, but with an onset weeks after the peak COVID-19 caseload, suggests MIS-C is a secondary consequence of SARS-CoV-2 infection. Indeed, over 70% of MIS-C patients test positive for serum antibodies against SARS-CoV-2 and test negative for the presence of viral RNA ( Cheung et al, 2020 ; Jones et al, 2020 ; Klocperk et al, 2020 ; Rauf et al ; Riphagen et al, 2020 ; Toubiana et al, 2020 ; Verdoni et al, 2020 ; Whittaker et al, 2020 ). Aside from this association, the pathophysiology of MIS-C remains largely unexplored.…”

Enfermedad de Kawasaki, enfermedad similar a Kawasaki y MIS-C asociado a COVID-19 en niños: revisión sistemática

“…Durante los últimos meses, desde el comienzo de la pandemia causada por el virus del Síndrome Respiratorio Agudo Severo tipo 2 (SARS-CoV-2), los datos epidemiológicos de muchos países informan de algunos niños con una presentación clínica similar a la de la enfermedad de Kawasaki (EK). Algunos de estos pacientes también desarrollaron una novedosa presentación clínica del síndrome inflamatorio multisistémico en niños (MIS-C, por sus siglas en inglés) 1 . En Londres, se describió un grupo de ocho niños con características clínicas de la enfermedad de Kawasaki, presumiblemente relacionadas con enfermedad por coronavirus 2019 (COVID- 19), que también se asociaba con shock.…”