Pulmonary hypertension associated with connective tissue disease

Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTDs). CTDs may cause PH due to several mechanisms; pulmonary arterial hypertension, associated interstitial lung disease, neuromuscular disease, and/or sleep disordered breathing leading to h...

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Main Authors: Srinivas Rajagopala, Molly Mary Thabah
Format: Article
Language:English
Published: SAGE Publishing 2017-01-01
Series:Indian Journal of Rheumatology
Subjects:
Online Access:http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=1;spage=38;epage=47;aulast=Rajagopala
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author Srinivas Rajagopala
Molly Mary Thabah
author_facet Srinivas Rajagopala
Molly Mary Thabah
author_sort Srinivas Rajagopala
collection DOAJ
description Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTDs). CTDs may cause PH due to several mechanisms; pulmonary arterial hypertension, associated interstitial lung disease, neuromuscular disease, and/or sleep disordered breathing leading to hypoxia, associated thromboembolic PH, and pulmonary venous hypertension due to left ventricular dysfunction. PH can be measured on echocardiography, but the gold standard for diagnosis is right heart catheterization. PH-specific therapy in addition to immunosuppression is the most common treatment used though data are scant. In this narrative review, we discuss the epidemiologic burden, clinical presentation, evaluation, and management of PH in CTDs.
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series Indian Journal of Rheumatology
spelling doaj-art-49af2fe7a36c4d01b13dfea705e549c42025-02-02T22:45:22ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012017-01-01121384710.4103/0973-3698.199124Pulmonary hypertension associated with connective tissue diseaseSrinivas RajagopalaMolly Mary ThabahPulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTDs). CTDs may cause PH due to several mechanisms; pulmonary arterial hypertension, associated interstitial lung disease, neuromuscular disease, and/or sleep disordered breathing leading to hypoxia, associated thromboembolic PH, and pulmonary venous hypertension due to left ventricular dysfunction. PH can be measured on echocardiography, but the gold standard for diagnosis is right heart catheterization. PH-specific therapy in addition to immunosuppression is the most common treatment used though data are scant. In this narrative review, we discuss the epidemiologic burden, clinical presentation, evaluation, and management of PH in CTDs.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=1;spage=38;epage=47;aulast=RajagopalaConnective tissue diseasemixed connective tissue diseaseprogressive systemic sclerosispulmonary hypertensionsystemic lupus erythrematosus
spellingShingle Srinivas Rajagopala
Molly Mary Thabah
Pulmonary hypertension associated with connective tissue disease
Indian Journal of Rheumatology
Connective tissue disease
mixed connective tissue disease
progressive systemic sclerosis
pulmonary hypertension
systemic lupus erythrematosus
title Pulmonary hypertension associated with connective tissue disease
title_full Pulmonary hypertension associated with connective tissue disease
title_fullStr Pulmonary hypertension associated with connective tissue disease
title_full_unstemmed Pulmonary hypertension associated with connective tissue disease
title_short Pulmonary hypertension associated with connective tissue disease
title_sort pulmonary hypertension associated with connective tissue disease
topic Connective tissue disease
mixed connective tissue disease
progressive systemic sclerosis
pulmonary hypertension
systemic lupus erythrematosus
url http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=1;spage=38;epage=47;aulast=Rajagopala
work_keys_str_mv AT srinivasrajagopala pulmonaryhypertensionassociatedwithconnectivetissuedisease
AT mollymarythabah pulmonaryhypertensionassociatedwithconnectivetissuedisease