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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation

Patient: Male, 56 Final Diagnosis: ANCA associated vasculitis Symptoms: Dyspnea Medication: — Clinical Procedure: — Specialty: Cardiology OBJECTIVE: Rare disease BACKGROUND: Granulomatosis with polyangiitis (GPA)/Wegener’s granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/...

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Autores principales: Guterbaum, Thomas Jeremy, Husic, Mirza, Voss, Anne, Dahl, Jordi Sanchez
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454581/
https://www.ncbi.nlm.nih.gov/pubmed/30930460
http://dx.doi.org/10.12659/AJCR.912693
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author Guterbaum, Thomas Jeremy
Husic, Mirza
Voss, Anne
Dahl, Jordi Sanchez
author_facet Guterbaum, Thomas Jeremy
Husic, Mirza
Voss, Anne
Dahl, Jordi Sanchez
author_sort Guterbaum, Thomas Jeremy
collection PubMed
description Patient: Male, 56 Final Diagnosis: ANCA associated vasculitis Symptoms: Dyspnea Medication: — Clinical Procedure: — Specialty: Cardiology OBJECTIVE: Rare disease BACKGROUND: Granulomatosis with polyangiitis (GPA)/Wegener’s granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss’ syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT: We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30–35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS: Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.
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spelling pubmed-64545812019-04-17 Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation Guterbaum, Thomas Jeremy Husic, Mirza Voss, Anne Dahl, Jordi Sanchez Am J Case Rep Articles Patient: Male, 56 Final Diagnosis: ANCA associated vasculitis Symptoms: Dyspnea Medication: — Clinical Procedure: — Specialty: Cardiology OBJECTIVE: Rare disease BACKGROUND: Granulomatosis with polyangiitis (GPA)/Wegener’s granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss’ syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT: We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30–35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS: Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered. International Scientific Literature, Inc. 2019-04-01 /pmc/articles/PMC6454581/ /pubmed/30930460 http://dx.doi.org/10.12659/AJCR.912693 Text en © Am J Case Rep, 2019 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Articles
Guterbaum, Thomas Jeremy
Husic, Mirza
Voss, Anne
Dahl, Jordi Sanchez
Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title_full Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title_fullStr Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title_full_unstemmed Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title_short Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
title_sort anti-neutrophil cytoplasmic antibody (anca) associated vasculitis causes aortic valve degeneration and severe aortic regurgitation
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454581/
https://www.ncbi.nlm.nih.gov/pubmed/30930460
http://dx.doi.org/10.12659/AJCR.912693
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