Role of intravenous immunoglobulin in the treatment of Kawasaki disease

Lo, Mindy S.; Newburger, Jane W.

doi:10.1111/1756-185x.13220

Cited by 59 publications

(52 citation statements)

References 48 publications

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“…It was noteworthy that 100% patients with neurological involvement admitted via the Neurology Department received non-standard IVIG treatment (1 g/kg for 2 days) due to suspected immune disease, while more than 50% of patients admitted via the Pediatric ICU and Infectious Disease Department received IVIG treatment at a dose of 500 mg/kg for an initial consideration of severe infectious disease. Substantial evidence has suggested that non-standard treatment could predispose KD patients to a higher risk of IVIG resistance [28][29][30][31]. However, a further multivariate logistic regression analysis failed to demonstrate neurological involvement as an independent risk factor for IVIG resistance in patients with KD.…”

Section: Discussionmentioning

confidence: 99%

Neurological involvement in Kawasaki disease: a retrospective study

et al. 2020

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Background: Kawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children. Neurological involvement is a known complication of KD, however, its association with KD severity remains elusive. We aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs). Methods: We retrospectively reviewed data from 1582 patients with KD between January 2013 and December 2017. Profiles of patients with neurological symptoms (group A, n = 80) were compared to those of gender-and admission date-matched patients without neurological involvement (group B, n = 512). Multivariate logistic regression analyses were performed to determine whether neurological involvement was significantly associated with IVIG resistance. Results: Neurological involvement was observed in 5.1% (80/1582) of patients with KD. The neurological manifestations were diffuse, presenting as headache (13/80, 16.3%), convulsions (14/80, 17.5%), somnolence (40/80, 50.1%), extreme irritability (21/80, 26.3%), signs of meningeal irritation (15/80, 18.8%), bulging fontanelles (7/80, 8.8%), and facial palsy (1/80, 1.3%). Neurological symptoms represented the initial and/or predominant manifestation in 47.5% (38/80) of patients with KD. The incidence of IVIG resistance and levels of inflammatory markers were higher in group A than in group B. However, neurological involvement was not an independent risk factor for IVIG resistance or CALs. Conclusion: Rates of neurological involvement were relatively low in patients with KD. Neurological involvement was associated with an increased risk of IVIG resistance and severe inflammatory burden. Our results highlight the need for pediatricians to recognize KD with neurological involvement and the importance of standard IVIG therapy. Trial registration: Retrospectively registered.

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Section: Discussionmentioning

confidence: 99%

Neurological involvement in Kawasaki disease: a retrospective study

et al. 2020

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“…Treatment of KD typically features intravenous immunoglobulin (IVIG) therapy (4). In fact, IVIG has markedly decreased the mortality rate in patients with acute KD.…”

Section: Introductionmentioning

confidence: 99%

Aetiological Significance of Infectious Stimuli in Kawasaki Disease

2019

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Kawasaki disease (KD) is a pediatric vasculitis syndrome that is often involves coronary artery lesions (e. g., coronary artery aneurysms). Although its causal factors and entire pathogenesis remain elusive, the available evidence indicates that the pathogenesis of KD is closely associated with dysregulation of immune responses to various viruses or microbes. In this short review, we address several essential aspects of the etiology of KD with respect to the immune response to infectious stimuli: 1) the role of viral infections, 2) the role of bacterial infections and the superantigen hypothesis, 3) involvement of innate immune response including pathogens/microbe-associated molecular patterns and complement pathways, and 4) the influence of genetic background on the response to infectious stimuli. Based on the clinical and experimental evidence, we discuss the possibility that a wide range of microbes and viruses could cause KD through common and distinct immune processes.

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“…The first implication of humoral immunity possibly being involved in KD was the response to immunoglobulin infusions. IVIG was started after success with preparations in Immune thrombocytopenic purpura and was shown to protect against cardiac involvement (142). After discovery that aneurysms are associated with KD, studies, including randomized controlled trials, supported a protective effect of IVIG (136).…”

Section: Genetic Implicationsmentioning

confidence: 99%

Antibodies and Immunity During Kawasaki Disease

Hicar

2020

Front. Cardiovasc. Med.

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The cause of Kawasaki disease (KD), the leading cause of acquired heart disease in children, is currently unknown. Epidemiology studies support that an infectious disease is involved in at least starting the inflammatory cascade set off during KD. Clues from epidemiology support that humoral immunity can have a protective effect. However, the role of the immune system, particularly of B cells and antibodies, in pathogenesis of KD is still unclear. Intravenous immunoglobulin (IVIG) and other therapies targeted at modulating inflammation can prevent development of coronary aneurysms. A number of autoantibody responses have been reported in children with KD and antibodies have been generated from aneurysmal plasma cell infiltrates. Recent reports show that children with KD have similar plasmablast responses as other children with infectious diseases, further supporting an infectious starting point. As ongoing studies are attempting to identify the etiology of KD through study of antibody responses, we sought to review the role of humoral immunity in KD pathogenesis, treatment, and recovery.

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Role of intravenous immunoglobulin in the treatment of Kawasaki disease

Cited by 59 publications

References 48 publications

Neurological involvement in Kawasaki disease: a retrospective study

Neurological involvement in Kawasaki disease: a retrospective study

Aetiological Significance of Infectious Stimuli in Kawasaki Disease

Antibodies and Immunity During Kawasaki Disease

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