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Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg–Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivi...

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Autores principales: Chai, Joshua T, McGrath, Sam, Lopez, Begoña, Dworakowski, Rafal
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177047/
https://www.ncbi.nlm.nih.gov/pubmed/31020153
http://dx.doi.org/10.1093/ehjcr/yty075
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author Chai, Joshua T
McGrath, Sam
Lopez, Begoña
Dworakowski, Rafal
author_facet Chai, Joshua T
McGrath, Sam
Lopez, Begoña
Dworakowski, Rafal
author_sort Chai, Joshua T
collection PubMed
description BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg–Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivity. CASE SUMMARY: Here, we report a highly unusual case of acute ST-elevation myocardial infarction in a young and fit man with no cardiovascular risk factors. His emergency coronary angiography revealed disproportionately severe widespread coronary artery disease. We describe the diagnostic challenges with emphasis on meticulous history-taking (deep hyponasal voice, anosmia, and childhood asthma), supported by timely blood markers (peripheral eosinophilia and raised CRP), and multi-modal imaging (severe paranasal sinusitis on cranial magnetic resonance imaging and multiple lung infiltrates with small patches of ground-glass appearance on thoracic computed tomography), to reach a diagnosis of EGPA coronary vasculitis with particular reference to the American College of Rheumatology EGPA classification. Importantly, with prompt immunosuppression, his coronary lesions resolved completely without the need of any surgical or percutaneous revascularisation. He remained well and asymptomatic on maintenance immunosuppressants at 1 year follow-up. DISCUSSION: This case highlighted the rare but recognized involvement of the coronary arteries in systemic EGPA vasculitis, which can sometimes mimic atherosclerotic coronary disease and acute coronary syndrome.
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spelling pubmed-61770472019-04-24 Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report Chai, Joshua T McGrath, Sam Lopez, Begoña Dworakowski, Rafal Eur Heart J Case Rep Case Reports BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg–Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivity. CASE SUMMARY: Here, we report a highly unusual case of acute ST-elevation myocardial infarction in a young and fit man with no cardiovascular risk factors. His emergency coronary angiography revealed disproportionately severe widespread coronary artery disease. We describe the diagnostic challenges with emphasis on meticulous history-taking (deep hyponasal voice, anosmia, and childhood asthma), supported by timely blood markers (peripheral eosinophilia and raised CRP), and multi-modal imaging (severe paranasal sinusitis on cranial magnetic resonance imaging and multiple lung infiltrates with small patches of ground-glass appearance on thoracic computed tomography), to reach a diagnosis of EGPA coronary vasculitis with particular reference to the American College of Rheumatology EGPA classification. Importantly, with prompt immunosuppression, his coronary lesions resolved completely without the need of any surgical or percutaneous revascularisation. He remained well and asymptomatic on maintenance immunosuppressants at 1 year follow-up. DISCUSSION: This case highlighted the rare but recognized involvement of the coronary arteries in systemic EGPA vasculitis, which can sometimes mimic atherosclerotic coronary disease and acute coronary syndrome. Oxford University Press 2018-09-21 /pmc/articles/PMC6177047/ /pubmed/31020153 http://dx.doi.org/10.1093/ehjcr/yty075 Text en © The Author(s) 2018. Published by Oxford University Press on behalf of the European Society of Cardiology. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Reports
Chai, Joshua T
McGrath, Sam
Lopez, Begoña
Dworakowski, Rafal
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title_full Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title_fullStr Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title_full_unstemmed Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title_short Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) masquerading as acute ST-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
title_sort eosinophilic granulomatosis with polyangiitis (churg–strauss syndrome) masquerading as acute st-elevation myocardial infarction with complete resolution after immunosuppressive therapy: a case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177047/
https://www.ncbi.nlm.nih.gov/pubmed/31020153
http://dx.doi.org/10.1093/ehjcr/yty075
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