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

Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmon...

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Autores principales:	Tselios K, Gladman DD, Urowitz MB
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2016
Materias:	systemic lupus erythematosus pulmonary arterial hypertension immunosuppressive Diseases of the musculoskeletal system RC925-935
Acceso en línea:	https://doaj.org/article/2be12be71c584846b503667374ac2088
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spelling	oai:doaj.org-article:2be12be71c584846b503667374ac20882021-12-02T03:08:31ZSystemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies1179-156Xhttps://doaj.org/article/2be12be71c584846b503667374ac20882016-12-01T00:00:00Zhttps://www.dovepress.com/systemic-lupus-erythematosus-and-pulmonary-arterial-hypertension-links-peer-reviewed-article-OARRRhttps://doaj.org/toc/1179-156XKonstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: 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. Keywords: systemic lupus erythematosus, pulmonary arterial hypertension, immunosuppressive, transthoracic echocardiogram, endothelin receptor antagonistsTselios KGladman DDUrowitz MBDove Medical Pressarticlesystemic lupus erythematosuspulmonary arterial hypertensionimmunosuppressiveDiseases of the musculoskeletal systemRC925-935ENOpen Access Rheumatology: Research and Reviews, Vol Volume 9, Pp 1-9 (2016)
institution	DOAJ
collection	DOAJ
language	EN
topic	systemic lupus erythematosus pulmonary arterial hypertension immunosuppressive Diseases of the musculoskeletal system RC925-935
spellingShingle	systemic lupus erythematosus pulmonary arterial hypertension immunosuppressive Diseases of the musculoskeletal system RC925-935 Tselios K Gladman DD Urowitz MB Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies
description	Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: 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. Keywords: systemic lupus erythematosus, pulmonary arterial hypertension, immunosuppressive, transthoracic echocardiogram, endothelin receptor antagonists
format	article
author	Tselios K Gladman DD Urowitz MB
author_facet	Tselios K Gladman DD Urowitz MB
author_sort	Tselios K
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
publisher	Dove Medical Press
publishDate	2016
url	https://doaj.org/article/2be12be71c584846b503667374ac2088
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_version_	1718401907725172736

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

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