Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition

We describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our...

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Main Authors: Kofi A. Mensah, Rajwardhan Yadav, Terence K. Trow, Cristina M. Brunet, Wassim H. Fares
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2015/328435
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author Kofi A. Mensah
Rajwardhan Yadav
Terence K. Trow
Cristina M. Brunet
Wassim H. Fares
author_facet Kofi A. Mensah
Rajwardhan Yadav
Terence K. Trow
Cristina M. Brunet
Wassim H. Fares
author_sort Kofi A. Mensah
collection DOAJ
description We describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our report highlights the variable course that SLE-associated PAH can take in the same patient and the implications of this for instituting the most effective treatment approach with each episode. This report also highlights the potential for SLE-associated PAH to present with life-threatening symptoms requiring critical care level interventions. We also describe evidence-based therapies, which can result in significant improvement in symptoms, function, and long-term outcomes.
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spelling doaj-art-ab8e7e85ddb94e24bbc9942b29e584932025-08-20T02:20:13ZengWileyCase Reports in Medicine1687-96271687-96352015-01-01201510.1155/2015/328435328435Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt RecognitionKofi A. Mensah0Rajwardhan Yadav1Terence K. Trow2Cristina M. Brunet3Wassim H. Fares4Section of Rheumatology, Department of Internal Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USASection of Rheumatology, Department of Internal Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USASection of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USASection of Rheumatology, Department of Internal Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USASection of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USAWe describe a critically ill young woman with systemic lupus erythematosus (SLE) presenting with circulatory shock, multiorgan dysfunction, and elevated right-sided heart pressures. She was found to have recurrent acute severe pulmonary arterial hypertension (PAH) in the setting of an SLE flare. Our report highlights the variable course that SLE-associated PAH can take in the same patient and the implications of this for instituting the most effective treatment approach with each episode. This report also highlights the potential for SLE-associated PAH to present with life-threatening symptoms requiring critical care level interventions. We also describe evidence-based therapies, which can result in significant improvement in symptoms, function, and long-term outcomes.http://dx.doi.org/10.1155/2015/328435
spellingShingle Kofi A. Mensah
Rajwardhan Yadav
Terence K. Trow
Cristina M. Brunet
Wassim H. Fares
Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
Case Reports in Medicine
title Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
title_full Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
title_fullStr Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
title_full_unstemmed Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
title_short Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition
title_sort lupus associated pulmonary arterial hypertension variable course and importance of prompt recognition
url http://dx.doi.org/10.1155/2015/328435
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