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Combined pulmonary fibrosis and emphysema: an increasingly recognized condition.

Dias OM, Baldi BG, Costa AN, Carvalho CR - J Bras Pneumol (2014 May-Jun)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Instituto do Coração, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.

ABSTRACT
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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CT scan of the chest of a 67-year-old female patient with combinedpulmonary fibrosis and emphysema, showing centrilobular and paraseptalemphysema in the upper lobes (A and B), as well as ground-glass opacities,traction bronchiectasis, and honeycombing in the lower lobes (C and D). Notean aspergilloma in one of the paraseptal bullae in the right upper lobe(black arrow, in B)
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f01: CT scan of the chest of a 67-year-old female patient with combinedpulmonary fibrosis and emphysema, showing centrilobular and paraseptalemphysema in the upper lobes (A and B), as well as ground-glass opacities,traction bronchiectasis, and honeycombing in the lower lobes (C and D). Notean aspergilloma in one of the paraseptal bullae in the right upper lobe(black arrow, in B)

Mentions: The mainstay of the diagnosis of CPFE, HRCT scans typically show centrilobular orparaseptal emphysema in the upper lobes, as well as reticular opacities, tractionbronchiectasis, septal thickening, ground-glass opacities, and honeycombing in thelower lobes(3) (Figures 1 and 2). Although UIP isthe most common CT pattern, some patients have ground-glass opacities that are moreextensive than expected for a UIP pattern and are therefore suggestive of nonspecificinterstitial pneumonia, RB-ILD, and even desquamative interstitialpneumonia.(3)


Combined pulmonary fibrosis and emphysema: an increasingly recognized condition.

Dias OM, Baldi BG, Costa AN, Carvalho CR - J Bras Pneumol (2014 May-Jun)

CT scan of the chest of a 67-year-old female patient with combinedpulmonary fibrosis and emphysema, showing centrilobular and paraseptalemphysema in the upper lobes (A and B), as well as ground-glass opacities,traction bronchiectasis, and honeycombing in the lower lobes (C and D). Notean aspergilloma in one of the paraseptal bullae in the right upper lobe(black arrow, in B)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4109203&req=5

f01: CT scan of the chest of a 67-year-old female patient with combinedpulmonary fibrosis and emphysema, showing centrilobular and paraseptalemphysema in the upper lobes (A and B), as well as ground-glass opacities,traction bronchiectasis, and honeycombing in the lower lobes (C and D). Notean aspergilloma in one of the paraseptal bullae in the right upper lobe(black arrow, in B)
Mentions: The mainstay of the diagnosis of CPFE, HRCT scans typically show centrilobular orparaseptal emphysema in the upper lobes, as well as reticular opacities, tractionbronchiectasis, septal thickening, ground-glass opacities, and honeycombing in thelower lobes(3) (Figures 1 and 2). Although UIP isthe most common CT pattern, some patients have ground-glass opacities that are moreextensive than expected for a UIP pattern and are therefore suggestive of nonspecificinterstitial pneumonia, RB-ILD, and even desquamative interstitialpneumonia.(3)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Instituto do Coração, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus