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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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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. |
format | Online Article Text |
id | pubmed-8825418 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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