Combinación de fibrosis pulmonar y enfisema

Portillo, Karina; Sánchez, Juan Roldán; Prat, Josep Morera

doi:10.1016/j.arbres.2010.06.011

Cited by 6 publications

(13 citation statements)

References 46 publications

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“…Laboratory animal studies have demonstrated that oxidative stress inducing inflammatory cell activation, elevated matrix metalloproteinase levels causing proteolytic activity, ( 17 , 18 ) and overexpression of other mediators, such as PDGF, ( 19 ) TNF-α, and TGF-β, ( 20 , 21 ) are potential pathways explaining the lesions that lead to emphysema and fibrosis. A study analyzing inflammatory mediators in BAL fluid from patients with IPF showed significantly higher concentrations of chemokine (C-X-C motif) ligand 5 and chemokine (C-X-C motif) ligand 8 in those with concomitant HRCT findings of emphysema.…”

Section: Introductionmentioning

confidence: 99%

“…( 3 , 33 , 34 ) The prevalence of PAH in CPFE patients varies from 47% to 90%, being considerably higher than that in patients with COPD or IPF alone. ( 18 ) Indeed, the five-year survival rate in a study involving CPFE patients was 25% in those with PAH (as measured by transthoracic echocardiography), being 75% in those without PAH. ( 28 ) In another study, the finding of severe PAH on echocardiography was associated with an increased risk of death.…”

Section: Introductionmentioning

confidence: 99%

“…Estudos em animais de laboratório demonstraram que o estresse oxidativo que induz a ativação de células inflamatórias, os níveis elevados de metaloproteinases da matriz que causam a atividade proteolítica ( 17 , 18 ) e a superexpressão de outros mediadores, tais como PDGF, ( 19 ) TNF-α e TGF-β, ( 20 , 21 ) são potenciais vias que explicam as lesões que levam a enfisema e fibrose. Um estudo em que se analisaram os mediadores inflamatórios no lavado broncoalveolar de pacientes com FPI mostrou concentrações significativamente maiores das quimiocinas CXCL5 e CXCL8 naqueles que concomitantemente apresentaram enfisema na TCAR.…”

Section: Introductionunclassified

“…( 3 , 33 , 34 ) A prevalência de HAP em pacientes com CFPE varia de 47% a 90%, sendo consideravelmente maior do que a observada em pacientes com DPOC ou FPI apenas. ( 18 ) De fato, a taxa de sobrevida em cinco anos em um estudo com pacientes com CFPE foi de 25% naqueles com HAP (medida por meio de ecocardiografia transtorácica) e de 75% naqueles sem HAP. (28) Em outro estudo, o achado ecocardiográfico de HAP grave relacionou-se com um aumento do risco de morte.…”

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Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

et al. 2014

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Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionunclassified

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Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

et al. 2014

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“…Lung tissue expresses high levels of caveolins, and dysfunctions have been linked to pulmonary hypertension associated with COPD and emphysema, interstitial fibrosis, and lung cancer. As a result, caveolin-1 may be another important mediator in the etiology of CPFE, and it undoubtedly deserves to be the subject of further investigations [43]. …”

Section: Pathogenesismentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease

Portillo

Morera

2012

Pulmonary Medicine

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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.

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