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Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome
AIMS : Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported. METHODS AND RESULTS : Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigate...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8088814/ https://www.ncbi.nlm.nih.gov/pubmed/33355356 http://dx.doi.org/10.1093/eurheartj/ehaa973 |
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author | Frustaci, Andrea Alfarano, Maria Verardo, Romina Agrati, Chiara Casetti, Rita Miraldi, Fabio Galea, Nicola Letizia, Claudio Chimenti, Cristina |
author_facet | Frustaci, Andrea Alfarano, Maria Verardo, Romina Agrati, Chiara Casetti, Rita Miraldi, Fabio Galea, Nicola Letizia, Claudio Chimenti, Cristina |
author_sort | Frustaci, Andrea |
collection | PubMed |
description | AIMS : Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported. METHODS AND RESULTS : Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigated with immunohistochemistry for toll-like receptor 4 (TLR4) and real-time polymerase chain reaction (PCR) for viral genomes. Serum samples were processed for anti-heart autoantibodies (Abs), IL-1β, IL-6, IL-8, tumour necrosis factor (TNF)-α. Identification of an immunologic pathway (including virus-negativity, TLR4-, and Ab-positivity) was followed by immunosuppression. Myocarditis-NCV cohort was followed for 6 months with 2D-echo and/or cardiac magnetic resonance and compared with 60 Myocarditis patients and 30 controls. Increase in left ventricular ejection fraction ≥10% was classified as response to therapy. Control endomyocardial biopsy followed the end of treatment. Twenty-six Myocarditis-NCV patients presented with heart failure; four with electrical instability. Cause of Myocarditis-NCV included infectious agents (10%) and immune-mediated causes (chest trauma 3%; drug hypersensitivity 7%; hypereosinophilic syndrome 3%; primary autoimmune diseases 33%, idiopathic 44%). Abs were positive in immune-mediated Myocarditis-NCV and virus-negative Myocarditis; Myocarditis-NCV patients with Ab+ presented autoreactivity in vessel walls. Toll-like receptor 4 was overexpressed in immune-mediated forms and poorly detectable in viral. Interleukin-1β was significantly higher in Myocarditis-NCV than Myocarditis, the former presenting 24% in-hospital mortality compared with 1.5% of Myocarditis cohort. Immunosuppression induced improvement of cardiac function in 88% of Myocarditis-NCV and 86% of virus-negative Myocarditis patients. CONCLUSION : Necrotizing coronary vasculitis is histologically detectable in 1.5% of Myocarditis. Necrotizing coronary vasculitis includes viral and immune-mediated causes. Intra-hospital mortality is 24%. The immunologic pathway is associated with beneficial response to immunosuppression. |
format | Online Article Text |
id | pubmed-8088814 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-80888142021-05-05 Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome Frustaci, Andrea Alfarano, Maria Verardo, Romina Agrati, Chiara Casetti, Rita Miraldi, Fabio Galea, Nicola Letizia, Claudio Chimenti, Cristina Eur Heart J Clinical Research AIMS : Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported. METHODS AND RESULTS : Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigated with immunohistochemistry for toll-like receptor 4 (TLR4) and real-time polymerase chain reaction (PCR) for viral genomes. Serum samples were processed for anti-heart autoantibodies (Abs), IL-1β, IL-6, IL-8, tumour necrosis factor (TNF)-α. Identification of an immunologic pathway (including virus-negativity, TLR4-, and Ab-positivity) was followed by immunosuppression. Myocarditis-NCV cohort was followed for 6 months with 2D-echo and/or cardiac magnetic resonance and compared with 60 Myocarditis patients and 30 controls. Increase in left ventricular ejection fraction ≥10% was classified as response to therapy. Control endomyocardial biopsy followed the end of treatment. Twenty-six Myocarditis-NCV patients presented with heart failure; four with electrical instability. Cause of Myocarditis-NCV included infectious agents (10%) and immune-mediated causes (chest trauma 3%; drug hypersensitivity 7%; hypereosinophilic syndrome 3%; primary autoimmune diseases 33%, idiopathic 44%). Abs were positive in immune-mediated Myocarditis-NCV and virus-negative Myocarditis; Myocarditis-NCV patients with Ab+ presented autoreactivity in vessel walls. Toll-like receptor 4 was overexpressed in immune-mediated forms and poorly detectable in viral. Interleukin-1β was significantly higher in Myocarditis-NCV than Myocarditis, the former presenting 24% in-hospital mortality compared with 1.5% of Myocarditis cohort. Immunosuppression induced improvement of cardiac function in 88% of Myocarditis-NCV and 86% of virus-negative Myocarditis patients. CONCLUSION : Necrotizing coronary vasculitis is histologically detectable in 1.5% of Myocarditis. Necrotizing coronary vasculitis includes viral and immune-mediated causes. Intra-hospital mortality is 24%. The immunologic pathway is associated with beneficial response to immunosuppression. Oxford University Press 2020-12-23 /pmc/articles/PMC8088814/ /pubmed/33355356 http://dx.doi.org/10.1093/eurheartj/ehaa973 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical Research Frustaci, Andrea Alfarano, Maria Verardo, Romina Agrati, Chiara Casetti, Rita Miraldi, Fabio Galea, Nicola Letizia, Claudio Chimenti, Cristina Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title | Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title_full | Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title_fullStr | Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title_full_unstemmed | Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title_short | Myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
title_sort | myocarditis-associated necrotizing coronary vasculitis: incidence, cause, and outcome |
topic | Clinical Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8088814/ https://www.ncbi.nlm.nih.gov/pubmed/33355356 http://dx.doi.org/10.1093/eurheartj/ehaa973 |
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