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

Lee, S J; Kim, Danbi; Kim, Beom Joon; Rhim, Jung-Woo; Lee, Soo‐Young; Jeong, Dae Chul

doi:10.4078/jrd.2023.0022

Cited by 7 publications

(11 citation statements)

References 15 publications

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“…The age of onset of MIS-C in children is older than for KD [ 104 , 105 , 106 ]. KD and MIS are currently considered to be distinct due to differences in patient age demographics and additional MIS-X symptoms (e.g., ventricle dysfunction, gastrointestinal, and neurological) [ 119 ]. Complement activation is seen in some MIS-C children with rapid improvement after IVIG treatment; however, this was not associated with detectable immune complexes [ 120 ].…”

Section: Discussionmentioning

confidence: 99%

VAERS Vasculitis Adverse Events Retrospective Study: Etiology Model of Immune Complexes Activating Fc Receptors in Kawasaki Disease and Multisystem Inflammatory Syndromes

Ricke,

Smith

2024

Life

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Background: Vasculitis diseases include Kawasaki disease (KD), Kawasaki disease shock syndrome (KDSS), Multisystem Inflammatory Syndrome (MIS), Henoch–Schönlein purpura (HS), or IgA vasculitis, and additional vasculitis diseases. These diseases are often preceded by infections or immunizations. Disease incidence rates are higher in children than in adults. These diseases have been extensively studied, but understanding of the disease etiology remains to be established. Objective: Many studies have failed to demonstrate an association between vasculitis diseases and vaccination; this study examines possible associations. Methods: Herein, the Vaccine Adverse Event Reporting System (VAERS) database is retrospectively examined for associations between vasculitis diseases and immunizations. Results: For some vaccines, the number of rare cases of KD, MIS, and HS are higher than the background rates. These rare cases are predicted to occur in individuals with (1) genetic risk factors with (2) antibody titer levels above the primary immune response level. Herein, the model of humoral immune response antibodies bound to antigens (pathogen or vaccine) creating immune complexes is proposed. These immune complexes are proposed to bind Fc receptors on immune cells and platelets, resulting in cell activation and the release of inflammatory molecules including histamine and serotonin. Immune complexes and inflammatory molecules including serotonin and histamine likely trigger vasculitis. Elevated serotonin and possibly histamine drive initial vasoconstrictions, disrupting blood flow. Increased blood flow pressure from cardiac capillary vasoconstrictions is predicted to trigger coronary artery aneurysms (CAA) or lesions (CAL) in some patients. For KDSS and MIS patients, these cardiac capillary vasoconstrictions are predicted to result in ischemia followed by ventricular dysfunction. Ongoing ischemia can result in long-term cardiac damage. Cases associated with pathogens are likely to have persistent infections triggering disease onset. Conclusion: The proposed model of immune complexes driving disease initial disease etiology by Fc receptor activation of immune cells and platelets, resulting in elevated histamine and serotonin levels, is testable and is consistent with disease symptoms and current treatments.

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Section: Discussionmentioning

confidence: 99%

VAERS Vasculitis Adverse Events Retrospective Study: Etiology Model of Immune Complexes Activating Fc Receptors in Kawasaki Disease and Multisystem Inflammatory Syndromes

Ricke,

Smith

2024

Life

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“…Table 5. Important characteristics of MIS-C [26], KDSS [13], and KD [28] a Modified from our previous study with permission [10]. b Data from [16].…”

Section: Discussionmentioning

confidence: 99%

“…Conjunctivitis and oropharyngeal inflammation appeared on the sixth day of hospitalization. Because KD-like features may not be present in the acute phase of MIS-C, and one-quarter of patients with MIS-C do not develop KD-like features throughout their disease [ 25 , 26 ], MIS-C should be suspected in patients presenting with unknown etiology of shock, heart failure, or gastrointestinal symptoms, even in the absence of KD-like features [ 10 , 27 ].…”

Section: Discussionmentioning

confidence: 99%

“…MIS-C is a serious complication that can occur two to eight weeks after SARS-CoV-2 infection and is characterized by infectious triggers, systemic inflammation, and organ dysfunction [ 7 ]. The presence of “KD-like features” in patients with MIS-C has received clinical attention, but the extent of organ dysfunction in patients with MIS-C is of greater importance as it directly affects the disease course and outcome [ 8 , 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

“…Because MIS-C and KD share KD-like features, many studies have been conducted to elucidate the relationship between MIS-C and KD [ 8 , 9 ]. Few studies have addressed the relationship between MIS-C and KDSS [ 10 , 14 ], even though MIS-C and KDSS share organ dysfunction in addition to KD-like features.…”

Section: Introductionmentioning

confidence: 99%

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Similarities and Differences between Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki Disease Shock Syndrome

Lee,

Kim,

Cho

et al. 2023

Children

Self Cite

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This study aimed to investigate the characteristics of COVID-19-associated multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease shock syndrome (KDSS) and to compare the similarities and differences between the two diseases. The incidence of KDSS and MIS-C was also estimated. Medical records of patients diagnosed with MIS-C or KDSS at four hospitals from January 2013 to December 2022 were retrospectively reviewed. Thirty-one patients were enrolled in the study in either an MIS-C group (n = 22) or a KDSS group (n = 9). The incidence of KDSS in KD was 0.8% (9/1095) and the incidence of MIS-C versus KD was 10.2% (22/216). Compared with the MIS-C group, the KDSS group had longer hospital stays and more severe systemic inflammation (e.g., anemia, elevated C-reactive protein, hypoalbuminemia, and pyuria) and organ dysfunction (e.g., number of involved organs, shock, vasoactive infusion, and intensive care unit admission). All patients in the MIS-C group, but none in the KDSS group, including two patients during the COVID-19 pandemic, had laboratory evidence of SARS-CoV-2 infection. MIS-C and KDSS shared demographic, clinical, and laboratory characteristics; organ dysfunction; treatment; and outcomes. Overall severity was more severe in patients with KDSS than in those with MIS-C. The most important difference between MIS-C and KDSS was whether SARS-CoV-2 was identified as an infectious trigger.

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Etiology Model of Kawasaki Disease and Multisystem Inflammatory Syndromes

Ricke,

Smith

2024

Preprint

View full text Add to dashboard Cite

Vasculitis diseases include Kawasaki Disease (KD), Kawasaki Disease Shock Syndrome (KDSS), Multisystem Inflammatory Syndromes (MIS), Henoch-Schönlein purpura (HS) or IgA Vasculitis, and additional vasculitis diseases. These diseases are often preceded by infections or immunizations. Disease incidence rates are higher in children than adults. These diseases have been extensively studied, but understanding of the disease etiology remains to be established. Many studies have failed to demonstrate an association between these vasculitis diseases and vaccination. Here, the Vaccine Adverse Event Reporting System (VAERS) is used to demonstrate that, in certain rare cases, the number of cases of KD, MIS, and HS is higher than background following vaccination. These rare cases are predicted to occur in individuals with genetic risk factors who produce antibody titer levels above primary immune response level. Herein, the model of humoral immune response antibodies bound to antigens (pathogen or vaccine) creating immune complexes is proposed. These immune complexes are proposed to bind Fc receptors on immune cells and platelets resulting in cell activation and release of inflammatory molecules including histamine and serotonin. Immune complexes and inflammatory molecules including serotonin and histamine likely trigger vasculitis. Elevated serotonin and likely histamine drive initial vasoconstrictions disrupting blood flow. Increased blood flow pressure from cardiac capillary vasoconstrictions is predicted to trigger coronary artery aneurysms in some patients. For KDSS and MIS patients, these cardiac capillary vasoconstrictions are predicted to result in ischemia followed by ventricular dysfunction. Ongoing ischemia can result in long-term cardiac damage. Cases associated with pathogens are likely to have persistent infections triggering disease onset.

show abstract

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

Cited by 7 publications

References 15 publications

VAERS Vasculitis Adverse Events Retrospective Study: Etiology Model of Immune Complexes Activating Fc Receptors in Kawasaki Disease and Multisystem Inflammatory Syndromes

VAERS Vasculitis Adverse Events Retrospective Study: Etiology Model of Immune Complexes Activating Fc Receptors in Kawasaki Disease and Multisystem Inflammatory Syndromes

Similarities and Differences between Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki Disease Shock Syndrome

Etiology Model of Kawasaki Disease and Multisystem Inflammatory Syndromes

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