J Rheum Dis.  2023 Oct;30(4):272-277. 10.4078/jrd.2023.0022.

Comparison of COVID-19-associated multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease shock syndrome: case reports and literature review

• Lee S
• Kim D
• Kim BJ
• Rhim JW
• Lee SY
• Jeong DC

• KMID: 2546244
• DOI: http://doi.org/10.4078/jrd.2023.0022

Abstract

Multisystem inflammatory syndrome in children (MIS-C) is a serious post-infectious complication of COVID-19 characterized by hyperinflammation and multi-organ dysfunction including shock. Shock is also seen in a severe form of Kawasaki disease (KD) called KD shock syndrome (KDSS). Here, we present one MIS-C and one KDSS case and compare similarities and differences between them. Both MIS-C (case 1) and KDSS (case 2) showed hyperinflammation, KD-related features, gastrointestinal problems, hypotension, and coagulopathy. The extent of systemic inflammation and organ dysfunction was more severe in KDSS than in MIS-C. Case 1 was diagnosed as MIS-C because SARS-CoV-2 was confirmed, and case 2 was diagnosed as KDSS because no pathogen was identified in microbiological studies. We believe that the most important difference between MIS-C and KDSS was whether SARS-CoV-2 was identified as an infectious trigger. Organ dysfunction is a hallmark of MIS-C and KDSS, but not KD, so MIS-C shares more clinical phenotypes with KDSS than with KD. Comparison of MIS-C and KDSS will be an interesting and important topic in the field of KD-like hyperinflammatory disease research.

Keyword

COVID-19; Multisystem inflammatory syndrome in children; Kawasaki disease shock syndrome; Kawasaki disease

Figure

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  Fig. 1 Echocardiography of a patient with Kawasaki disease shock syndrome (case 2) at 1 week after discharge. The apical four-chamber view (A) shows an aneurysm of 4.1 mm in the left anterior descending (LAD) and the parasternal short-axis view (B) shows an aneurysmal dilatation of 3.6 mm in the proximal LAD. Each ventricular function is normal, with no additional pericardiac effusion.

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  Fig. 2 The relationship between KDSS and MIS-C similar to that between pathogen-unidentified septic shock and pneumococcal septic shock. (A) When pneumococcus is identified in a patient with septic shock, the patient is diagnosed with ‘pneumococcus-confirmed septic shock.’ (B) When SARS-CoV-2 is identified in a patient with KDSS, the patient should be diagnosed with ‘SARS-Co-V-2-confirmed KDSS (i.e., MIS-C).’ KD: Kawasaki disease, KDSS: Kawasaki disease shock syndrome, MIS-C: multisystem inflammatory syndrome in children, SARS-CoV-2: severe acute respiratory syndrome coronavirus 2.

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