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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...

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Autores principales: Frustaci, Andrea, Alfarano, Maria, Verardo, Romina, Agrati, Chiara, Casetti, Rita, Miraldi, Fabio, Galea, Nicola, Letizia, Claudio, Chimenti, Cristina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
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.
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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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