Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg)...

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Main Authors: Tselios, Konstantinos, Gladman, Dafna D, Urowitz, Murray B
Format: Online
Language:English
Published: Dove Medical Press 2016
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5191623/
id pubmed-5191623
recordtype oai_dc
spelling pubmed-51916232017-01-04 Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies Tselios, Konstantinos Gladman, Dafna D Urowitz, Murray B Review Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively. Dove Medical Press 2016-12-20 /pmc/articles/PMC5191623/ /pubmed/28053559 http://dx.doi.org/10.2147/OARRR.S123549 Text en © 2017 Tselios et al. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author Tselios, Konstantinos
Gladman, Dafna D
Urowitz, Murray B
spellingShingle Tselios, Konstantinos
Gladman, Dafna D
Urowitz, Murray B
Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
author_facet Tselios, Konstantinos
Gladman, Dafna D
Urowitz, Murray B
author_sort Tselios, Konstantinos
title Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
title_short Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
title_full Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
title_fullStr Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
title_full_unstemmed Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
title_sort systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
description Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.
publisher Dove Medical Press
publishDate 2016
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5191623/
_version_ 1613832825871532032

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