Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development

Kawasaki disease (KD) is an acute systemic vasculitis that occurs predominantly in children under 5 years of age. Despite much study, the etiology of KD remains unknown. However, epidemiological and immunological data support the hygiene hypothesis as a possible etiology. It is thought that more ste...

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Autor principal: Jong-Keuk Lee
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Publicado: MDPI AG 2021
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spelling oai:doaj.org-article:ab799210005d4cc98a99db55301fb03a2021-11-25T17:55:29ZHygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development10.3390/ijms2222123341422-00671661-6596https://doaj.org/article/ab799210005d4cc98a99db55301fb03a2021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12334https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Kawasaki disease (KD) is an acute systemic vasculitis that occurs predominantly in children under 5 years of age. Despite much study, the etiology of KD remains unknown. However, epidemiological and immunological data support the hygiene hypothesis as a possible etiology. It is thought that more sterile or clean modern living environments due to increased use of sanitizing agents, antibiotics, and formula feeding result in a lack of immunological challenges, leading to defective or dysregulated B cell development, accompanied by low IgG and high IgE levels. A lack of B cell immunity may increase sensitivity to unknown environmental triggers that are nonpathogenic in healthy individuals. Genetic studies of KD show that all of the KD susceptibility genes identified by genome-wide association studies are involved in B cell development and function, particularly in early B cell development (from the pro-B to pre-B cell stage). The fact that intravenous immunoglobulin is an effective therapy for KD supports this hypothesis. In this review, I discuss clinical, epidemiological, immunological, and genetic studies showing that the etiopathogenesis of KD in infants and toddlers can be explained by the hygiene hypothesis, and particularly by defects or dysregulation during early B cell development.Jong-Keuk LeeMDPI AGarticleKawasaki diseasehygiene hypothesisB cellsintravenous immunoglobulinBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12334, p 12334 (2021)
institution DOAJ
collection DOAJ
language EN
topic Kawasaki disease
hygiene hypothesis
B cells
intravenous immunoglobulin
Biology (General)
QH301-705.5
Chemistry
QD1-999
spellingShingle Kawasaki disease
hygiene hypothesis
B cells
intravenous immunoglobulin
Biology (General)
QH301-705.5
Chemistry
QD1-999
Jong-Keuk Lee
Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
description Kawasaki disease (KD) is an acute systemic vasculitis that occurs predominantly in children under 5 years of age. Despite much study, the etiology of KD remains unknown. However, epidemiological and immunological data support the hygiene hypothesis as a possible etiology. It is thought that more sterile or clean modern living environments due to increased use of sanitizing agents, antibiotics, and formula feeding result in a lack of immunological challenges, leading to defective or dysregulated B cell development, accompanied by low IgG and high IgE levels. A lack of B cell immunity may increase sensitivity to unknown environmental triggers that are nonpathogenic in healthy individuals. Genetic studies of KD show that all of the KD susceptibility genes identified by genome-wide association studies are involved in B cell development and function, particularly in early B cell development (from the pro-B to pre-B cell stage). The fact that intravenous immunoglobulin is an effective therapy for KD supports this hypothesis. In this review, I discuss clinical, epidemiological, immunological, and genetic studies showing that the etiopathogenesis of KD in infants and toddlers can be explained by the hygiene hypothesis, and particularly by defects or dysregulation during early B cell development.
format article
author Jong-Keuk Lee
author_facet Jong-Keuk Lee
author_sort Jong-Keuk Lee
title Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
title_short Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
title_full Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
title_fullStr Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
title_full_unstemmed Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development
title_sort hygiene hypothesis as the etiology of kawasaki disease: dysregulation of early b cell development
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/ab799210005d4cc98a99db55301fb03a
work_keys_str_mv AT jongkeuklee hygienehypothesisastheetiologyofkawasakidiseasedysregulationofearlybcelldevelopment
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