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Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report

Takayasu arteritis (TAK) is a rare primary systemic inflammatory vasculopathy. It is classified as a large-vessel vasculitis and is known to cause inflammatory aneurysms and vascular stenosis. Vascular Ehlers–Danlos syndrome (vEDS) is an autosomal dominant condition known to cause multiple aneurysms...

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Autores principales: Hashimoto, Kyota, Sakai, Ryota, Shibata, Akiko, Okada, Yusuke, Yoshinaga, Syoichi, Kurasawa, Takahiko, Kondo, Tsuneo, Amano, Koichi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8825418/
https://www.ncbi.nlm.nih.gov/pubmed/35155630
http://dx.doi.org/10.3389/fcvm.2022.805505
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author Hashimoto, Kyota
Sakai, Ryota
Shibata, Akiko
Okada, Yusuke
Yoshinaga, Syoichi
Kurasawa, Takahiko
Kondo, Tsuneo
Amano, Koichi
author_facet Hashimoto, Kyota
Sakai, Ryota
Shibata, Akiko
Okada, Yusuke
Yoshinaga, Syoichi
Kurasawa, Takahiko
Kondo, Tsuneo
Amano, Koichi
author_sort Hashimoto, Kyota
collection PubMed
description Takayasu arteritis (TAK) is a rare primary systemic inflammatory vasculopathy. It is classified as a large-vessel vasculitis and is known to cause inflammatory aneurysms and vascular stenosis. Vascular Ehlers–Danlos syndrome (vEDS) is an autosomal dominant condition known to cause multiple aneurysms and arterial dissection at a young age owing to a mutation in the gene for type III collagen, COL3A1. Here, we present a case of TAK associated with vEDS with the development of multi-organ infarction of the brain, kidney, and spleen owing to multiple arterial aneurysms and stenosis of the internal carotid artery. The patient was successfully treated using anti-inflammatory agents, glucocorticoids, and tocilizumab with the addition of interventional radiology. In our case, a high inflammatory response led to vasculitis being the main cause of the disease with concurrent vEDS. When patients develop multiple aneurysms, stenosis, and dissections leading to multiple organ infarctions, a systemic differential diagnosis to consider concurrent vasculitis syndrome and non-inflammatory vasculopathy, including hereditary disorders, is important even with time constraints.
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spelling pubmed-88254182022-02-10 Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report Hashimoto, Kyota Sakai, Ryota Shibata, Akiko Okada, Yusuke Yoshinaga, Syoichi Kurasawa, Takahiko Kondo, Tsuneo Amano, Koichi Front Cardiovasc Med Cardiovascular Medicine Takayasu arteritis (TAK) is a rare primary systemic inflammatory vasculopathy. It is classified as a large-vessel vasculitis and is known to cause inflammatory aneurysms and vascular stenosis. Vascular Ehlers–Danlos syndrome (vEDS) is an autosomal dominant condition known to cause multiple aneurysms and arterial dissection at a young age owing to a mutation in the gene for type III collagen, COL3A1. Here, we present a case of TAK associated with vEDS with the development of multi-organ infarction of the brain, kidney, and spleen owing to multiple arterial aneurysms and stenosis of the internal carotid artery. The patient was successfully treated using anti-inflammatory agents, glucocorticoids, and tocilizumab with the addition of interventional radiology. In our case, a high inflammatory response led to vasculitis being the main cause of the disease with concurrent vEDS. When patients develop multiple aneurysms, stenosis, and dissections leading to multiple organ infarctions, a systemic differential diagnosis to consider concurrent vasculitis syndrome and non-inflammatory vasculopathy, including hereditary disorders, is important even with time constraints. Frontiers Media S.A. 2022-01-26 /pmc/articles/PMC8825418/ /pubmed/35155630 http://dx.doi.org/10.3389/fcvm.2022.805505 Text en Copyright © 2022 Hashimoto, Sakai, Shibata, Okada, Yoshinaga, Kurasawa, Kondo and Amano. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Cardiovascular Medicine
Hashimoto, Kyota
Sakai, Ryota
Shibata, Akiko
Okada, Yusuke
Yoshinaga, Syoichi
Kurasawa, Takahiko
Kondo, Tsuneo
Amano, Koichi
Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title_full Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title_fullStr Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title_full_unstemmed Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title_short Concurrent Takayasu Arteritis and Vascular Ehlers–Danlos Syndrome: A Case Report
title_sort concurrent takayasu arteritis and vascular ehlers–danlos syndrome: a case report
topic Cardiovascular Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8825418/
https://www.ncbi.nlm.nih.gov/pubmed/35155630
http://dx.doi.org/10.3389/fcvm.2022.805505
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