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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2021-11-01
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author | Jong-Keuk Lee |
author_facet | Jong-Keuk Lee |
author_sort | Jong-Keuk Lee |
collection | DOAJ |
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. |
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spelling | doaj.art-ab799210005d4cc98a99db55301fb03a2023-11-22T23:40:39ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-11-0122221233410.3390/ijms222212334Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell DevelopmentJong-Keuk Lee0Asan Medical Center, Asan Institute for Life Sciences, University of Ulsan College of Medicine, Seoul 05505, KoreaKawasaki 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.https://www.mdpi.com/1422-0067/22/22/12334Kawasaki diseasehygiene hypothesisB cellsintravenous immunoglobulin |
spellingShingle | Jong-Keuk Lee Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development International Journal of Molecular Sciences Kawasaki disease hygiene hypothesis B cells intravenous immunoglobulin |
title | 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_short | 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 |
topic | Kawasaki disease hygiene hypothesis B cells intravenous immunoglobulin |
url | https://www.mdpi.com/1422-0067/22/22/12334 |
work_keys_str_mv | AT jongkeuklee hygienehypothesisastheetiologyofkawasakidiseasedysregulationofearlybcelldevelopment |