The impact of emphysema in pulmonary fibrosis
Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is cha...
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doaj-5e2e109a5c534b64925a3fc1e1d4ae6f2020-11-25T01:29:39ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172013-06-012212815315710.1183/09059180.00000813The impact of emphysema in pulmonary fibrosis Vincent CottinSeveral groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.http://err.ersjournals.com/content/22/128/153.full.pdf+htmlCombined pulmonary fibrosis and emphysemaemphysemaidiopathic pulmonary fibrosispulmonary hypertension |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Vincent Cottin |
spellingShingle |
Vincent Cottin The impact of emphysema in pulmonary fibrosis European Respiratory Review Combined pulmonary fibrosis and emphysema emphysema idiopathic pulmonary fibrosis pulmonary hypertension |
author_facet |
Vincent Cottin |
author_sort |
Vincent Cottin |
title |
The impact of emphysema in pulmonary fibrosis |
title_short |
The impact of emphysema in pulmonary fibrosis |
title_full |
The impact of emphysema in pulmonary fibrosis |
title_fullStr |
The impact of emphysema in pulmonary fibrosis |
title_full_unstemmed |
The impact of emphysema in pulmonary fibrosis |
title_sort |
impact of emphysema in pulmonary fibrosis |
publisher |
European Respiratory Society |
series |
European Respiratory Review |
issn |
0905-9180 1600-0617 |
publishDate |
2013-06-01 |
description |
Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE. |
topic |
Combined pulmonary fibrosis and emphysema emphysema idiopathic pulmonary fibrosis pulmonary hypertension |
url |
http://err.ersjournals.com/content/22/128/153.full.pdf+html |
work_keys_str_mv |
AT vincentcottin theimpactofemphysemainpulmonaryfibrosis AT vincentcottin impactofemphysemainpulmonaryfibrosis |
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