Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

Dias, Olívia Meira; Baldi, Bruno Guedes; Costa, André Nathan; Carvalho, Carlos Roberto Ribeiro

doi:10.1590/s1806-37132014000300014

Cited by 19 publications

(25 citation statements)

References 46 publications

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“…22 This may delay diagnosis, as spirometry is the most easily accessible pulmonary function test. 27 Although some studies reported lower values of DLco in patients with CPFE compared to in IPF, 24 including in our study and a study by Jacob et al 28 , similar values were observed in both groups (IPF and CPFE). Interestingly, Kco was lower in the CPFE group.…”

Section: Discussionsupporting

confidence: 82%

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Anselmo

Costa

Rodrigues

et al. 2020

Preprint

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Background Combined pulmonary fibrosis and emphysema (CPFE) is an entity characterized by the presence of emphysema in upper lobes and fibrosis in lower lobes. Due to the presence of the two diseases concomitantly, it may be difficult to diagnose. This study aims at a better understanding of this entity and proposes biological markers (functional and biochemical) that help in this characterization. Methods A prospective, observational, cross-sectional study was carried out at a reference center. Pulmonary function tests (spirometry, CO-diffusion capacity, plethysmography and single-maneuver nitrogen washout test - SBWN 2 ) and biochemical markers (periostin, mucin-16, PDGF-BB and TGF-β 1 ) were measured in groups of patients: idiopathic pulmonary fibrosis, CPFE and chronic obstructive pulmonary disease (COPD). Results Variables derived from SBWN 2 - closing volume (CV) / vital capacity (VC) (%) and closing capacity (CC) / total lung capacity (TLC) (%) - were found to be higher in the CPFE group compared to the Idiopathic pulmonary fibrosis (IPF) group (CV/VC%: 0.25 (0.12 – 11.01) and 13.05 (0.21 – 20.73); p = 0.005; CC/TLC%: 30.1 (22.4 – 37.47) and 33.69 (32.05 – 41.98); p = 0.03, respectively). Periostin was higher in the CPFE group than in the other groups [CPFE: 66.74 (45.21 – 90.5), IPF: 43.81 (31.97 – 56.18), COPD: 40.08 (20.66 – 50.81); p = 0.0002], and mucin-16 was higher in the IPF group than in the CPFE group [CPFE: 13.59 (4.16 – 28.16); IPF: 71.94 (40.46 - 164); COPD: 25.85 (9.27 – 30.29); p = 0.02]. Conclusions Findings show that CPFE presents different functional and biochemical characteristics than IPF, including higher CV/VC%, CC/TLC% and periostin, whereas mucin-16 was higher in the IPF.

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Section: Discussionsupporting

confidence: 82%

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Anselmo

Costa

Rodrigues

et al. 2020

Preprint

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“…This is explained by the cohabitation of the two types of lesions present in CPFE: emphysema and fibrosis. While emphysema causes hyperinflation, obstruction of airways, and greater lung compliance, fibrosis causes restrictive effects, traction to support the small airways and prevent them from collapsing, and a decrease in lung compliance 46. Therefore, CPFE patients have shown a faster decrease in forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) than patients with IPF only, and a higher decrease in vital capacity (VC) and FVC compared to patients with COPD alone 53 , 54…”

Section: Discussionmentioning

confidence: 99%

Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers

Hirano¹,

Targueta²,

Campos³

et al. 2017

Autops. Case Rep.

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In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.

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“…HRCT scanning is an essential part for diagnosing CPFE. Anyway, the relative contributions of pathological characteristics vary among patients with CPFE [8]. Diagnosis of CPFE on HRCT is still the certain method to assess the degree of emphysema and fibrosis, which may make the progress effective and accurate [9].…”

Section: Discussionmentioning

confidence: 99%

A New Variant of Combined Pulmonary Fibrosis and Emphysema from Long-Term High-Dose of Glucocorticoid Therapy: A Case Report

Zeng¹,

Duan²,

Dai³

2017

CRCM

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Recent studies have described the combination of both pulmonary emphysema and idiopathic interstitial lung disease (ILDs) by means of high-resolution computed axial tomography (HRCT). Definition of this syndrome was first named by Cottin as combined pulmonary fibrosis and emphysema (CPFE). Functional and radiological findings have showed that these patients are suffering from severe breathlessness, but whose pulmonary functional tests revealed no signs of obstruction, normal static lung volumes, and depressed DLco, most with a history of smoking [1] [2]. The radiological and endoscopic studies especially show that these patients have both areas of upper-lobe predominant emphysema and lesions compatible with fibrosis in both lung bases [3]. No prior research has reported any cases of such condition in person with no prior history of smoking as well as long-term high-dose of glucocorticoid therapy. In this case report, we discuss the presentation, diagnosis, and management of a 53-year-old non-smoker with increasing shortness of breath with a long-term high-dose of glucocorticoid therapy discovered to have an abnormal variant or presentation of CPFE. The cause of disease was attributed to a certain history of smoking in most studies; other potential risk factors have yet to be properly analyzed. This clinical report features a special case about the problem and solution surrounding this issue.

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Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

Cited by 19 publications

References 46 publications

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Periostin and closing volume in combined pulmonary fibrosis and emphysema

Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers

A New Variant of Combined Pulmonary Fibrosis and Emphysema from Long-Term High-Dose of Glucocorticoid Therapy: A Case Report

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