Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease
Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnorma...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Pulmonary Medicine |
| Online Access: | http://dx.doi.org/10.1155/2012/867870 |
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| author | Karina Portillo Josep Morera |
| author_facet | Karina Portillo Josep Morera |
| author_sort | Karina Portillo |
| collection | DOAJ |
| description | Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco) and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches. |
| format | Article |
| id | doaj-art-147f36a248244e17b1cf7a6ed16b9193 |
| institution | Kabale University |
| issn | 2090-1836 2090-1844 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Medicine |
| spelling | doaj-art-147f36a248244e17b1cf7a6ed16b91932025-02-03T05:43:53ZengWileyPulmonary Medicine2090-18362090-18442012-01-01201210.1155/2012/867870867870Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung DiseaseKarina Portillo0Josep Morera1Pulmonary Department, Hospital Germans Trias i Pujol, Carretera de Canyet s/n, 08916 Badalona, Barcelona, SpainPulmonary Department, Hospital Germans Trias i Pujol, Carretera de Canyet s/n, 08916 Badalona, Barcelona, SpainCombined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco) and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.http://dx.doi.org/10.1155/2012/867870 |
| spellingShingle | Karina Portillo Josep Morera Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease Pulmonary Medicine |
| title | Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease |
| title_full | Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease |
| title_fullStr | Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease |
| title_full_unstemmed | Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease |
| title_short | Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease |
| title_sort | combined pulmonary fibrosis and emphysema syndrome a new phenotype within the spectrum of smoking related interstitial lung disease |
| url | http://dx.doi.org/10.1155/2012/867870 |
| work_keys_str_mv | AT karinaportillo combinedpulmonaryfibrosisandemphysemasyndromeanewphenotypewithinthespectrumofsmokingrelatedinterstitiallungdisease AT josepmorera combinedpulmonaryfibrosisandemphysemasyndromeanewphenotypewithinthespectrumofsmokingrelatedinterstitiallungdisease |