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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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Related in: MedlinePlus

TC de tórax de uma paciente de 67 anos de idade com combinação defibrose pulmonar e enfisema, mostrando enfisema centrolobular eparasseptal nos lobos superiores (A e B), bem como opacidades em vidrofosco, bronquiectasias de tração e faveolamento nos lobos inferiores (C eD). Nota-se um aspergiloma em uma das bolhas parasseptais no lobosuperior direito (seta preta, em B).
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f03: TC de tórax de uma paciente de 67 anos de idade com combinação defibrose pulmonar e enfisema, mostrando enfisema centrolobular eparasseptal nos lobos superiores (A e B), bem como opacidades em vidrofosco, bronquiectasias de tração e faveolamento nos lobos inferiores (C eD). Nota-se um aspergiloma em uma das bolhas parasseptais no lobosuperior direito (seta preta, em B).

Mentions: A TCAR constitui a base do diagnóstico da CFPE e tipicamente mostra enfisemacentrolobular ou parasseptal nos lobos superiores, bem como opacidadesreticulares, bronquiectasias de tração, espessamento septal, opacidades em vidrofosco e faveolamento nos lobos inferiores(3) (Figuras 1 e 2). Embora a PIU seja o padrão tomográfico maiscomum, alguns pacientes apresentam opacidades em vidro fosco que são mais extensasdo que o esperado para o padrão de PIU e que, portanto, sugerem a presença depneumonia intersticial não específica, DPI-BR e mesmo pneumonia intersticialdescamativa.(3)


Combined pulmonary fibrosis and emphysema: an increasingly recognized condition.

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

TC de tórax de uma paciente de 67 anos de idade com combinação defibrose pulmonar e enfisema, mostrando enfisema centrolobular eparasseptal nos lobos superiores (A e B), bem como opacidades em vidrofosco, bronquiectasias de tração e faveolamento nos lobos inferiores (C eD). Nota-se um aspergiloma em uma das bolhas parasseptais no lobosuperior direito (seta preta, em B).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: TC de tórax de uma paciente de 67 anos de idade com combinação defibrose pulmonar e enfisema, mostrando enfisema centrolobular eparasseptal nos lobos superiores (A e B), bem como opacidades em vidrofosco, bronquiectasias de tração e faveolamento nos lobos inferiores (C eD). Nota-se um aspergiloma em uma das bolhas parasseptais no lobosuperior direito (seta preta, em B).
Mentions: A TCAR constitui a base do diagnóstico da CFPE e tipicamente mostra enfisemacentrolobular ou parasseptal nos lobos superiores, bem como opacidadesreticulares, bronquiectasias de tração, espessamento septal, opacidades em vidrofosco e faveolamento nos lobos inferiores(3) (Figuras 1 e 2). Embora a PIU seja o padrão tomográfico maiscomum, alguns pacientes apresentam opacidades em vidro fosco que são mais extensasdo que o esperado para o padrão de PIU e que, portanto, sugerem a presença depneumonia intersticial não específica, DPI-BR e mesmo pneumonia intersticialdescamativa.(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