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Screening for pulmonary arterial hypertension in systemic sclerosis

Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, ea...

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Autores principales: Weatherald, Jason, Montani, David, Jevnikar, Mitja, Jaïs, Xavier, Savale, Laurent, Humbert, Marc
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488700/
https://www.ncbi.nlm.nih.gov/pubmed/31366460
http://dx.doi.org/10.1183/16000617.0023-2019
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author Weatherald, Jason
Montani, David
Jevnikar, Mitja
Jaïs, Xavier
Savale, Laurent
Humbert, Marc
author_facet Weatherald, Jason
Montani, David
Jevnikar, Mitja
Jaïs, Xavier
Savale, Laurent
Humbert, Marc
author_sort Weatherald, Jason
collection PubMed
description Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due to suboptimal visualisation or insufficient tricuspid regurgitation. Other options for screening include the DETECT algorithm or the use of a combination of pulmonary function testing (forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio) and N-terminal-pro-brain natriuretic peptide levels. Symptomatic patients, those with an elevated tricuspid regurgitation velocity on echocardiogram with or without secondary echocardiographic features of PAH, and those who screen positive on the DETECT or other pulmonary function test algorithms should undergo right heart catheterisation. Exercise echocardiography or cardiopulmonary exercise testing, nailfold capillaroscopy and molecular biomarkers are promising but, as yet, unproven potential options. Future screening studies should employ systematic catheterisation to define the true predictive values for PAH.
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spelling pubmed-94887002022-11-14 Screening for pulmonary arterial hypertension in systemic sclerosis Weatherald, Jason Montani, David Jevnikar, Mitja Jaïs, Xavier Savale, Laurent Humbert, Marc Eur Respir Rev Review Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due to suboptimal visualisation or insufficient tricuspid regurgitation. Other options for screening include the DETECT algorithm or the use of a combination of pulmonary function testing (forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio) and N-terminal-pro-brain natriuretic peptide levels. Symptomatic patients, those with an elevated tricuspid regurgitation velocity on echocardiogram with or without secondary echocardiographic features of PAH, and those who screen positive on the DETECT or other pulmonary function test algorithms should undergo right heart catheterisation. Exercise echocardiography or cardiopulmonary exercise testing, nailfold capillaroscopy and molecular biomarkers are promising but, as yet, unproven potential options. Future screening studies should employ systematic catheterisation to define the true predictive values for PAH. European Respiratory Society 2019-07-31 /pmc/articles/PMC9488700/ /pubmed/31366460 http://dx.doi.org/10.1183/16000617.0023-2019 Text en Copyright ©ERS 2019. https://creativecommons.org/licenses/by-nc/4.0/This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
spellingShingle Review
Weatherald, Jason
Montani, David
Jevnikar, Mitja
Jaïs, Xavier
Savale, Laurent
Humbert, Marc
Screening for pulmonary arterial hypertension in systemic sclerosis
title Screening for pulmonary arterial hypertension in systemic sclerosis
title_full Screening for pulmonary arterial hypertension in systemic sclerosis
title_fullStr Screening for pulmonary arterial hypertension in systemic sclerosis
title_full_unstemmed Screening for pulmonary arterial hypertension in systemic sclerosis
title_short Screening for pulmonary arterial hypertension in systemic sclerosis
title_sort screening for pulmonary arterial hypertension in systemic sclerosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488700/
https://www.ncbi.nlm.nih.gov/pubmed/31366460
http://dx.doi.org/10.1183/16000617.0023-2019
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