Multi-System Inflammatory Syndrome in a Child Mimicking Kawasaki Disease

J Trop Pediatr. 2021 Jul 2;67(3):fmaa060. doi: 10.1093/tropej/fmaa060.

Abstract

There have been recent reports of children presenting with severe multi-system hyperinflammatory syndrome resembling Kawasaki disease (KD) during current COVID-19 pandemic. Exact pathophysiology is unknown, however, most of the children have multi-organ dysfunction and respiratory system involvement is less common compared to adults. These patients have certain characteristic laboratory parameters different from those seen in children with KD. However, only limited literature is available at present for identification and management of such patients. We report a young girl who presented with fever, rash and other manifestations mimicking classic KD and fulfilling the case definitions for pediatric multi-system inflammatory syndrome. She had lymphopenia, thrombocytopenia and hyponatremia in the absence of macrophage activation syndrome, similar to that seen in patients reported from UK and Italy. Clinical manifestations resolved and laboratory parameters improved with intravenous immunoglobulin and corticosteroids. Early recognition is important to administer immunomodulatory therapy which may be life saving for these patients.

Keywords: COVID-19; Kawasaki disease; MIS-C; PIMS.

Plain language summary

Several cases of a severe multi-system inflammatory syndrome have been reported in children during the current COVID-19 pandemic. Clinical manifestations may resemble Kawasaki disease (KD) which is the most common childhood vasculitis. A 7-year-old-girl presented with fever, rash and abdominal pain. Examination showed maculopapular rash over lower limbs, back, right ear, trunk and abdomen; erythema and swelling over bilateral upper eyelids; conjunctival injection; reddened lips and erythema over palms and soles. She had lymphopenia, thrombocytopenia, with elevated erythrocyte sedimentation rate, C-reactive protein (CRP), pro-brain natriuretic peptide and interleukin-6 (IL-6). Real-time polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was negative. Serology, however, could not be performed due to unavailability. Macrophage activation syndrome was ruled out with normal ferritin and triglyceride with raised fibrinogen level. Echocardiography showed normal coronary diameters. Child received meropenem, intravenous immunoglobulin, aspirin and methylprednisolone. She improved with resolution of fever, decrease in CRP, increase in platelet and lymphocyte count. Index child had features similar to those reported from UK and Italy: features of KD, abdominal pain, lymphopenia, thrombocytopenia, elevated IL-6 and myocardial dysfunction with no significant respiratory involvement. Pediatricians should be aware of such uncommon presentation in children to initiate early treatment with immunomodulatory therapy.

MeSH terms

  • COVID-19*
  • Child
  • Female
  • Humans
  • Mucocutaneous Lymph Node Syndrome* / diagnosis
  • Mucocutaneous Lymph Node Syndrome* / drug therapy
  • Pandemics
  • SARS-CoV-2
  • Systemic Inflammatory Response Syndrome

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related