Prevalence and progression of combined pulmonary fibrosis and emphysema in asymptomatic smokers: A case-control study

Chae, Kum Ju; Jin, Gong Yong; Han, Young Min; Kim, Yong Seek; Chon, Su Bin; Kwon, Keun Sang; Choi, Hye Mi; Lynch, David A.

doi:10.1007/s00330-015-3617-3

Cited by 23 publications

(20 citation statements)

References 23 publications

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“…Regarding prognosis, although in IPF the annual rate of decline in FVC, DLCO or the CPI are known to be independent predictors of survival, in patients with CPFE survival is strongly related to the decline of FEV 1 [48]. Furthermore, CPFE progresses more rapidly in active smokers compared to patients that have quit smoking [97]. Finally, finger clubbing seems to be also an independent predictor of mortality [64].…”

Section: Prognosismentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Papaioannou

Κostikas

Manali

et al. 2016

Respiratory Medicine

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Combined pulmonary fibrosis and emphysema (CPFE) is a clinical entity characterized by the coexistence of upper lobe emphysema and lower lobe fibrosis. Patients with this condition experience severe dyspnea and impaired gas exchange with preserved lung volumes. The diagnosis of the CPFE syndrome is based on HRCT imaging, showing the coexistence of emphysema and pulmonary fibrosis both in varying extent and locations within the lung parenchyma. Individual genetic background seem to predispose to the development of the disease. The risk of the development of pulmonary hypertension in patients with CPFE is high and related to poor prognosis. CPFE patients also present a high risk of lung cancer. Mortality is significant in patients with CPFE and median survival is reported between 2.1 and 8.5 years. Currently, no specific recommendations are available regarding the management of patients with CPFE. In this review we provide information on the existing knowledge on CPFE regarding the pathophysiology, clinical manifestations, imaging, complications, possible therapeutic interventions and prognosis of the disease.

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Section: Prognosismentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Papaioannou

Κostikas

Manali

et al. 2016

Respiratory Medicine

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“…In patients with COPD, the cessation of smoking reduces the risk of exacerbation [ 51 ] and subsequent mortality, even in patients with severe disease [ 52 ]. Furthermore, continuous smoking affects the progression of disease in patients with CPFE more strongly than former smoking does [ 53 ]. Therefore, the cessation of smoking is considered to essential for patients with CPFE.…”

Section: Discussionmentioning

confidence: 99%

Objective quantitative multidetector computed tomography assessments in patients with combined pulmonary fibrosis with emphysema: Relationship with pulmonary function and clinical events

et al. 2020

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Background Combined pulmonary fibrosis with emphysema (CPFE) is a clinically meaningful syndrome characterized by coexisting upper-lobe emphysema and lower-lobe interstitial fibrosis. However, ambiguous diagnostic criteria and, particularly, the absence of objective methods to quantify emphysematous/fibrotic lesions in patients with CPFE confound the interpretation of the pathophysiology of this syndrome. We analyzed the relationship between objectively quantified computed tomography (CT) measurements and the results of pulmonary function testing (PFT) and clinical events in CPFE patients. Materials and methods We enrolled 46 CPFE patients who underwent CT and PFT. The extent of emphysematous lesions was obtained by calculating the percent of low attenuation area (%LAA). The extent of fibrotic lesions was calculated as the percent of high attenuation area (%HAA). %LAA and %HAA values were combined to yield the percent of abnormal area (%AA). We assessed the relationships between CT parameters and other clinical indices, including PFT results. Multivariate analysis was performed to examine the association between the CT parameters and clinical events. Results A greater negative correlation with percent predicted diffusing capacity of the lung for carbon monoxide (DL CO %predicted) existed for %AA (r = -0.73, p < 0.001) than for %LAA or %HAA alone. The %HAA value was inversely correlated with percent predicted forced vital capacity (r = -0.48, p < 0.001), percent predicted total lung capacity (r = -0.48, p < 0.01), and DL CO %predicted (r = -0.47, p < 0.01). Multivariate logistic regression analysis found that %AA showed the strongest association with hospitalization events (odds ratio = 1.20, 95% confidence interval = 1.01–1.54, p = 0.029). Conclusion Quantitative CT measurements reflected deterioration in pulmonary function and were associated with hospitalization in patients with CPFE. This approach could serve as a useful method to determine the extent of lung morphology, pathophysiology, and the clinical course of patients with CPFE.

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“…On the other hand, mortality was similar in patients with CPFE and IPF in a study that defined CPFE as ≥10% emphysema. We believe that these inconsistent results may be affected by the imprecise differentiation of CPFE from IPF, as well as the heterogeneous study populations involved [ 17 ]. We only included patients with pathologically proven UIP and SRIF which exhibited emphysema with alveolar septal widening by collagen deposition without evidence of UIP.…”

Section: Discussionmentioning

confidence: 99%

“…The CT findings were interpreted on the basis of the recommendations of the nomenclature committee of the Fleischner Society [ 14 ]. Visual assessment was performed by modification of prior methods [ 15 – 17 ]. The extent of emphysema was estimated by using a six-point scale for each lobe as follows: 0 (no emphysema), 0.5 (trivial, <5%), 1 (mild, 5–25%), 2 (moderate, 26–50%), 3 (marked, 51–75%), or 4 (severe, >75%).…”

Section: Methodsmentioning

confidence: 99%

Differentiating Smoking-Related Interstitial Fibrosis (SRIF) from Usual Interstitial Pneumonia (UIP) with Emphysema Using CT Features Based on Pathologically Proven Cases

et al. 2016

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ObjectiveTo differentiate smoking-related interstitial fibrosis (SRIF) from usual interstitial pneumonia (UIP) with emphysema on CT in combined pulmonary fibrosis and emphysema (CPFE) patients.Materials and MethodsThis study was approved by the institutional review board and informed consent was waived. We included 65 patients who underwent lung biopsy under the suspicion of UIP pattern on HRCT, and after radiologic-pathologic correlation, they were divided into three groups: UIP without emphysema (n = 30), UIP with emphysema (n = 26), and SRIF (n = 9). The quantitative extent of emphysema in the entire lung was visually assessed and fibrotic patterns were qualitatively analyzed based on six characteristics (asymmetry, juxta-subpleural sparing, emphysema beside the honeycombing area, absence of ground grass attenuation/reticulation in honeycombing area, inhomogeneous honeycombing, and absence of honeycombing in the upper lobes). Kaplan-Meier analysis was used for survival analysis, and logistic regression with a receiver operating characteristic curve was used to predict the possibility of SRIF.ResultsIn qualitative analysis of fibrotic patterns, SRIF tended to exhibit more than three of six fibrotic features, whereas UIP with emphysema demonstrated about two of these characteristics (p = 0.035). In addition, SRIF had a higher extent of emphysema than UIP with emphysema when they have same amount of fibrosis (p = 0.014). In patients with SRIF, 5-year survival rate was 85.7%, while it was 40.7% in UIP with emphysema patients (p = 0.035).ConclusionFibrotic CT patterns and survival rate differed between SRIF and UIP with emphysema among CPFE patients, which explains the variable prognosis of CPFE.

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Prevalence and progression of combined pulmonary fibrosis and emphysema in asymptomatic smokers: A case-control study

Abstract: • The prevalence of CPFE in asymptomatic smokers is 3.1 % (63/2,016). • Progression of CPFE is associated with smoking status. • 3.5 years of follow-up period would be needed to identify CPFE progression.

Cited by 23 publications

References 23 publications

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Objective quantitative multidetector computed tomography assessments in patients with combined pulmonary fibrosis with emphysema: Relationship with pulmonary function and clinical events

Differentiating Smoking-Related Interstitial Fibrosis (SRIF) from Usual Interstitial Pneumonia (UIP) with Emphysema Using CT Features Based on Pathologically Proven Cases

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