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Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management
BACKGROUND: Combined idiopathic pulmonary fibrosis (IPF) with pulmonary emphysema (CPFE) is a syndrome with a characteristic presentation of upper lobe emphysema and lower lobe fibrosis. While CPFE is a strong determinant of secondary precapillary pulmonary hypertension (PH), there is limited eviden...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2013
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3633020/ https://www.ncbi.nlm.nih.gov/pubmed/23734851 http://dx.doi.org/10.1186/1756-0500-6-S1-S2 |
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author | Cottin, Vincent |
author_facet | Cottin, Vincent |
author_sort | Cottin, Vincent |
collection | PubMed |
description | BACKGROUND: Combined idiopathic pulmonary fibrosis (IPF) with pulmonary emphysema (CPFE) is a syndrome with a characteristic presentation of upper lobe emphysema and lower lobe fibrosis. While CPFE is a strong determinant of secondary precapillary pulmonary hypertension (PH), there is limited evidence regarding the management of patients with CPFE and PH. CASE PRESENTATION: A 63 year-old male presented in 2006 with dyspnoea on exertion having quit smoking in 2003. Clinical examination, together with high resolution computed tomography, bronchoalveolar lavage, and echocardiographic assessments, suggested a diagnosis of CPFE without PH. In 2007, the patient received intravenous cyclophosphamide, N-acetylcysteine, and short-term anticoagulation treatment. Due to remission of acute exacerbations, the patient received triple combination therapy (prednisone, N-acetylcysteine and azathioprine). Upon progressive clinical worsening, long-term supplemental oxygen therapy was initiated in 2009. Repeated right heart catheterisation in 2011 confirmed PH and worsening pulmonary haemodynamics, and off-label ambrisentan therapy was initiated. Dyspnoea remained at follow-up, although significant haemodynamic improvement was observed. CONCLUSION: CFPE is a distinct but under-recognized and common syndrome with a characteristic presentation. Further studies are needed to ascertain the etiology, morbidity, and mortality of CPEF with or without PH, and to evaluate novel management options. |
format | Online Article Text |
id | pubmed-3633020 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-36330202013-04-25 Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management Cottin, Vincent BMC Res Notes Case Report BACKGROUND: Combined idiopathic pulmonary fibrosis (IPF) with pulmonary emphysema (CPFE) is a syndrome with a characteristic presentation of upper lobe emphysema and lower lobe fibrosis. While CPFE is a strong determinant of secondary precapillary pulmonary hypertension (PH), there is limited evidence regarding the management of patients with CPFE and PH. CASE PRESENTATION: A 63 year-old male presented in 2006 with dyspnoea on exertion having quit smoking in 2003. Clinical examination, together with high resolution computed tomography, bronchoalveolar lavage, and echocardiographic assessments, suggested a diagnosis of CPFE without PH. In 2007, the patient received intravenous cyclophosphamide, N-acetylcysteine, and short-term anticoagulation treatment. Due to remission of acute exacerbations, the patient received triple combination therapy (prednisone, N-acetylcysteine and azathioprine). Upon progressive clinical worsening, long-term supplemental oxygen therapy was initiated in 2009. Repeated right heart catheterisation in 2011 confirmed PH and worsening pulmonary haemodynamics, and off-label ambrisentan therapy was initiated. Dyspnoea remained at follow-up, although significant haemodynamic improvement was observed. CONCLUSION: CFPE is a distinct but under-recognized and common syndrome with a characteristic presentation. Further studies are needed to ascertain the etiology, morbidity, and mortality of CPEF with or without PH, and to evaluate novel management options. BioMed Central 2013-04-16 /pmc/articles/PMC3633020/ /pubmed/23734851 http://dx.doi.org/10.1186/1756-0500-6-S1-S2 Text en Copyright © 2013 Cottin; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Cottin, Vincent Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title | Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title_full | Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title_fullStr | Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title_full_unstemmed | Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title_short | Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
title_sort | clinical case: combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3633020/ https://www.ncbi.nlm.nih.gov/pubmed/23734851 http://dx.doi.org/10.1186/1756-0500-6-S1-S2 |
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