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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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Formato: | Texto |
Lenguaje: | English |
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BioMed Central|1
2007
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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. |
format | Text |
id | pubmed-2072890 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2007 |
publisher | BioMed Central|1 |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT baughmanrobertp pulmonaryhypertensionassociatedwithsarcoidosis |