Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation

Pulmonary arterial hypertension (PAH) is an uncommon manifestation of systemic lupus erythematosus (SLE), affecting about 0.5% to 23.3% of the population worldwide. The causes of PAH associated with SLE are multifactorial. While it is generally associated with a full-blown picture of SLE, it may rar...

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Autores principales: Kamath, Sangita D, Ahmed, Tauheed, Upadhyay, Ajatshatru, Agarwal, Vijay
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Internal Medicine
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10284723/
https://www.ncbi.nlm.nih.gov/pubmed/37362506
http://dx.doi.org/10.7759/cureus.39359
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author Kamath, Sangita D
Ahmed, Tauheed
Upadhyay, Ajatshatru
Agarwal, Vijay
author_facet Kamath, Sangita D
Ahmed, Tauheed
Upadhyay, Ajatshatru
Agarwal, Vijay
author_sort Kamath, Sangita D
collection PubMed
description Pulmonary arterial hypertension (PAH) is an uncommon manifestation of systemic lupus erythematosus (SLE), affecting about 0.5% to 23.3% of the population worldwide. The causes of PAH associated with SLE are multifactorial. While it is generally associated with a full-blown picture of SLE, it may rarely be the presenting manifestation of the disease. We describe the case of a middle-aged woman who presented with features of severe PAH due to SLE. She was treated with vasodilators and immunosuppression (steroids and mycophenolate mofetil), with a partial response to treatment at six months follow-up.
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spelling pubmed-102847232023-06-23 Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation Kamath, Sangita D Ahmed, Tauheed Upadhyay, Ajatshatru Agarwal, Vijay Cureus Internal Medicine Pulmonary arterial hypertension (PAH) is an uncommon manifestation of systemic lupus erythematosus (SLE), affecting about 0.5% to 23.3% of the population worldwide. The causes of PAH associated with SLE are multifactorial. While it is generally associated with a full-blown picture of SLE, it may rarely be the presenting manifestation of the disease. We describe the case of a middle-aged woman who presented with features of severe PAH due to SLE. She was treated with vasodilators and immunosuppression (steroids and mycophenolate mofetil), with a partial response to treatment at six months follow-up. Cureus 2023-05-22 /pmc/articles/PMC10284723/ /pubmed/37362506 http://dx.doi.org/10.7759/cureus.39359 Text en Copyright © 2023, Kamath et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Kamath, Sangita D
Ahmed, Tauheed
Upadhyay, Ajatshatru
Agarwal, Vijay
Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title_full Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title_fullStr Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title_full_unstemmed Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title_short Pulmonary Arterial Hypertension (PAH) as the Initial Manifestation of Systematic Lupus Erythematosus (SLE): A Rare Presentation
title_sort pulmonary arterial hypertension (pah) as the initial manifestation of systematic lupus erythematosus (sle): a rare presentation
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10284723/
https://www.ncbi.nlm.nih.gov/pubmed/37362506
http://dx.doi.org/10.7759/cureus.39359
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