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Pulmonary hypertension associated with sarcoidosis

Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organi...

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Detalles Bibliográficos
Autor principal: Baughman, Robert P
Formato: Texto
Lenguaje:English
Publicado: BioMed Central|1 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2072890/
https://www.ncbi.nlm.nih.gov/pubmed/17767746
http://dx.doi.org/10.1186/ar2192
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author Baughman, Robert P
author_facet Baughman, Robert P
author_sort Baughman, Robert P
collection PubMed
description Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of endothelin (ET)-1 in plasma and bronchoalveolar lavage of some sarcoid patients suggest that ET-1 may be driving pulmonary fibrosis and sarcoidosis-associated pulmonary hypertension. Although a relationship between raised levels of ET-1 and clinical phenotype is yet to be identified, early evidence from studies of ET-1 blockade with drugs such as bosentan is encouraging. Such therapy possibly could be combined with standard anti-inflammatory agents to improve outcome.
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spelling pubmed-20728902007-11-10 Pulmonary hypertension associated with sarcoidosis Baughman, Robert P Arthritis Res Ther Review Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of endothelin (ET)-1 in plasma and bronchoalveolar lavage of some sarcoid patients suggest that ET-1 may be driving pulmonary fibrosis and sarcoidosis-associated pulmonary hypertension. Although a relationship between raised levels of ET-1 and clinical phenotype is yet to be identified, early evidence from studies of ET-1 blockade with drugs such as bosentan is encouraging. Such therapy possibly could be combined with standard anti-inflammatory agents to improve outcome. BioMed Central|1 2007 2007-08-15 /pmc/articles/PMC2072890/ /pubmed/17767746 http://dx.doi.org/10.1186/ar2192 Text en Copyright © 2007 BioMed Central Ltd
spellingShingle Review
Baughman, Robert P
Pulmonary hypertension associated with sarcoidosis
title Pulmonary hypertension associated with sarcoidosis
title_full Pulmonary hypertension associated with sarcoidosis
title_fullStr Pulmonary hypertension associated with sarcoidosis
title_full_unstemmed Pulmonary hypertension associated with sarcoidosis
title_short Pulmonary hypertension associated with sarcoidosis
title_sort pulmonary hypertension associated with sarcoidosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2072890/
https://www.ncbi.nlm.nih.gov/pubmed/17767746
http://dx.doi.org/10.1186/ar2192
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