2013
DOI: 10.1586/ers.12.80
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Combined pulmonary fibrosis and emphysema

Abstract: The advent of computed tomography permitted recognition of the coexistence of pulmonary fibrosis and emphysema (CPFE). Emphysema is usually encountered in the upper lobes preceding fibrosis of the lower lobes, and patients are smokers, predominantly male, with distinct physiologic profile characterized by preserved lung volumes and markedly reduced diffusion capacity. Actually, the term CPFE is reserved for the coexistence of any type and grade of radiological pulmonary emphysema and the idiopathic usual inter… Show more

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Cited by 47 publications
(60 citation statements)
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References 94 publications
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“…23,24 IPF with CPFE syndrome is also associated with a heavy smoking history, as demonstrated in Table 1 of the current study. In general, clinical features of CPFE are characterized as follows: 1) % FVC and FEV 1 /FVC are preserved, 2) DLCO is markedly decreased and 3) CPFE is complicated with PH and lung cancer.…”
Section: Discussionsupporting
confidence: 58%
“…23,24 IPF with CPFE syndrome is also associated with a heavy smoking history, as demonstrated in Table 1 of the current study. In general, clinical features of CPFE are characterized as follows: 1) % FVC and FEV 1 /FVC are preserved, 2) DLCO is markedly decreased and 3) CPFE is complicated with PH and lung cancer.…”
Section: Discussionsupporting
confidence: 58%
“…Se ha propuesto una predisposición genética en los pacientes que desarrollan combinación de fibrosis pulmonar y enfisema (17). En todas las series de pacientes con dicha combinación se describe asociación entre la presencia de enfermedad y antecedente de tabaquismo.…”
Section: Fisiopatologíaunclassified
“…En todas las series de pacientes con dicha combinación se describe asociación entre la presencia de enfermedad y antecedente de tabaquismo. En aquellos con enfisema y fibrosis pulmonar se considera que el estrés oxidativo inducido por el tabaco y sus productos de degradación (como la nicotina), desempeña un papel importante en la fisiopatología mediante la amplificación de la expresión de genes inflamatorios que codifican citoquinas, quemoquinas, mediadores, receptores y otras proteínas inflamatorias (17,18). Uno de los mediadores inflamatorios descritos, cuya expresión se induce por la nicotina, es el factor de necrosis tumoral alfa (TNFɑ), cuyos niveles se hallan elevados (sobreexpresión mediada por el promotor de la proteína C del surfactante) en ratones con combinación de fibrosis pulmonar y enfisema inducida (19,20).…”
Section: Fisiopatologíaunclassified
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