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Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure

Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from pr...

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Autores principales: Ghannam, Malik, Manousakis, Georgios
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7724079/
https://www.ncbi.nlm.nih.gov/pubmed/33324322
http://dx.doi.org/10.3389/fneur.2020.571716
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author Ghannam, Malik
Manousakis, Georgios
author_facet Ghannam, Malik
Manousakis, Georgios
author_sort Ghannam, Malik
collection PubMed
description Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Serologic testing showed positive 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) IgG antibodies, and muscle biopsy showed necrotizing myopathy. No alternative explanation for heart failure was found. Despite immunotherapy and symptomatic treatment, she died from multiorgan failure. Our study suggests that heart failure in anti HMGCR myopathy may not be as rare as previously thought, and therefore early cardiac evaluation should be considered in patients with this diagnosis, to minimize morbidity and mortality.
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spelling pubmed-77240792020-12-14 Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure Ghannam, Malik Manousakis, Georgios Front Neurol Neurology Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Serologic testing showed positive 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) IgG antibodies, and muscle biopsy showed necrotizing myopathy. No alternative explanation for heart failure was found. Despite immunotherapy and symptomatic treatment, she died from multiorgan failure. Our study suggests that heart failure in anti HMGCR myopathy may not be as rare as previously thought, and therefore early cardiac evaluation should be considered in patients with this diagnosis, to minimize morbidity and mortality. Frontiers Media S.A. 2020-11-25 /pmc/articles/PMC7724079/ /pubmed/33324322 http://dx.doi.org/10.3389/fneur.2020.571716 Text en Copyright © 2020 Ghannam and Manousakis. http://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 Neurology
Ghannam, Malik
Manousakis, Georgios
Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title_full Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title_fullStr Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title_full_unstemmed Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title_short Case Report: Immune Mediated Necrotizing Myopathy With IgG Antibodies to 3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase (HMGCR) May Present With Acute Systolic Heart Failure
title_sort case report: immune mediated necrotizing myopathy with igg antibodies to 3-hydroxy-3-methylglutaryl-coenzyme a reductase (hmgcr) may present with acute systolic heart failure
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7724079/
https://www.ncbi.nlm.nih.gov/pubmed/33324322
http://dx.doi.org/10.3389/fneur.2020.571716
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