Cargando…

Kawasaki disease: pathophysiology and insights from mouse models

Kawasaki disease is an acute febrile illness and systemic vasculitis of unknown aetiology that predominantly afflicts young children, causes coronary artery aneurysms and can result in long-term cardiovascular sequelae. Kawasaki disease is the leading cause of acquired heart disease among children i...

Descripción completa

Detalles Bibliográficos
Autores principales: Noval Rivas, Magali, Arditi, Moshe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7250272/
https://www.ncbi.nlm.nih.gov/pubmed/32457494
http://dx.doi.org/10.1038/s41584-020-0426-0
_version_ 1783538737093804032
author Noval Rivas, Magali
Arditi, Moshe
author_facet Noval Rivas, Magali
Arditi, Moshe
author_sort Noval Rivas, Magali
collection PubMed
description Kawasaki disease is an acute febrile illness and systemic vasculitis of unknown aetiology that predominantly afflicts young children, causes coronary artery aneurysms and can result in long-term cardiovascular sequelae. Kawasaki disease is the leading cause of acquired heart disease among children in the USA. Coronary artery aneurysms develop in some untreated children with Kawasaki disease, leading to ischaemic heart disease and myocardial infarction. Although intravenous immunoglobulin (IVIG) treatment reduces the risk of development of coronary artery aneurysms, some children have IVIG-resistant Kawasaki disease and are at increased risk of developing coronary artery damage. In addition, the lack of specific diagnostic tests and biomarkers for Kawasaki disease make early diagnosis and treatment challenging. The use of experimental mouse models of Kawasaki disease vasculitis has considerably improved our understanding of the pathology of the disease and helped characterize the cellular and molecular immune mechanisms contributing to cardiovascular complications, in turn leading to the development of innovative therapeutic approaches. Here, we outline the pathophysiology of Kawasaki disease and summarize and discuss the progress gained from experimental mouse models and their potential therapeutic translation to human disease.
format Online
Article
Text
id pubmed-7250272
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher Nature Publishing Group UK
record_format MEDLINE/PubMed
spelling pubmed-72502722020-05-27 Kawasaki disease: pathophysiology and insights from mouse models Noval Rivas, Magali Arditi, Moshe Nat Rev Rheumatol Review Article Kawasaki disease is an acute febrile illness and systemic vasculitis of unknown aetiology that predominantly afflicts young children, causes coronary artery aneurysms and can result in long-term cardiovascular sequelae. Kawasaki disease is the leading cause of acquired heart disease among children in the USA. Coronary artery aneurysms develop in some untreated children with Kawasaki disease, leading to ischaemic heart disease and myocardial infarction. Although intravenous immunoglobulin (IVIG) treatment reduces the risk of development of coronary artery aneurysms, some children have IVIG-resistant Kawasaki disease and are at increased risk of developing coronary artery damage. In addition, the lack of specific diagnostic tests and biomarkers for Kawasaki disease make early diagnosis and treatment challenging. The use of experimental mouse models of Kawasaki disease vasculitis has considerably improved our understanding of the pathology of the disease and helped characterize the cellular and molecular immune mechanisms contributing to cardiovascular complications, in turn leading to the development of innovative therapeutic approaches. Here, we outline the pathophysiology of Kawasaki disease and summarize and discuss the progress gained from experimental mouse models and their potential therapeutic translation to human disease. Nature Publishing Group UK 2020-05-26 2020 /pmc/articles/PMC7250272/ /pubmed/32457494 http://dx.doi.org/10.1038/s41584-020-0426-0 Text en © Springer Nature Limited 2020 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Review Article
Noval Rivas, Magali
Arditi, Moshe
Kawasaki disease: pathophysiology and insights from mouse models
title Kawasaki disease: pathophysiology and insights from mouse models
title_full Kawasaki disease: pathophysiology and insights from mouse models
title_fullStr Kawasaki disease: pathophysiology and insights from mouse models
title_full_unstemmed Kawasaki disease: pathophysiology and insights from mouse models
title_short Kawasaki disease: pathophysiology and insights from mouse models
title_sort kawasaki disease: pathophysiology and insights from mouse models
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7250272/
https://www.ncbi.nlm.nih.gov/pubmed/32457494
http://dx.doi.org/10.1038/s41584-020-0426-0
work_keys_str_mv AT novalrivasmagali kawasakidiseasepathophysiologyandinsightsfrommousemodels
AT arditimoshe kawasakidiseasepathophysiologyandinsightsfrommousemodels