IPF and CPFE—The Two Different Entities or Two Different Presentations of the Same Disease?

Kwiatkowska, Sylwia

doi:10.5603/arm.a2017.0049

Cited by 5 publications

(3 citation statements)

References 42 publications

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“…Most reported cases of CPFE share similar characteristics, including relatively preserved spirometric values, decreased DLCO, and PH, and DLCO values have been noted to be lower in the CPFE patients than in the IPF patients [7,17]. Similarly, in the present study, patients with CPFE recorded better spirometric values, which can be attributed in part to emphysema-related hyperinflation [18]. Fibrosis and emphysema have cumulative effects on impaired gas exchange, linked to reduced vascular surface area and pulmonary capillary blood volume plus alveolar membrane thickening [7].…”

Section: Discussionsupporting

confidence: 80%

Prognostic Significance of Red Cell Distribution Width in Idiopathic Pulmonary Fibrosis and Combined Pulmonary Fibrosis Emphysema

et al. 2020

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Objective: The red cell distribution width (RDW) is an inexpensive, readily available prognostic indicator of several diseases. RDW has been assessed as a prognostic biomarker in patients with idiopathic pulmonary fibrosis (IPF) in only one study; furthermore, the relationship between the RDW and combined pulmonary fibrosis emphysema (CPFE) has yet to be reported. Subjects and Methods: This single-center study was conducted between January 2015 and December 2018 in the Atatürk Chest Diseases and Chest Surgery Education and Research Hospital. Baseline characteristics, laboratory results, and survival status of patients were recorded. Results: The RDW value was significantly higher in the CPFE group than in the IPF group (median [IQR 25–75]; 16.8 [15.5–19] vs. 15.3 [13.7–16.8], p = 0.028). High RDW values were correlated with carbon monoxide diffusion capacity (DLCO) (r: −0.653 p = 0.001), 6-minute walking test (6MWT) distance (r: −0.361 p = 0.017), arterial partial oxygen pressure (PaO2) (r: −0.692 p < 0.001), and systolic pulmonary arterial pressure (SPAP) (r: 0.349 p = 0.022) in patients with fibrotic lung disease. The RDW value was significantly higher in the exitus group than in the survivors (median [IQR 25–75]; 18.4 [15.4–19] vs. 15.2 [13.5–17.2], p = 0.016). A univariate Cox regression analysis identified DLCO, SPAP, PaO2, and RDW as potential covariates of mortality. In a multivariate analysis, the DLCO (HR 1.21, 95% CI 1.11–1.47, p = 0.012) and RDW level (HR 1.65, 95% CI 1.09–2.47, p = 0.023) remained independent predictors of mortality. Conclusion: High RDW values appear to be a simple prognostic factor in patients with IPF or CPFE.

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Section: Discussionsupporting

confidence: 80%

Prognostic Significance of Red Cell Distribution Width in Idiopathic Pulmonary Fibrosis and Combined Pulmonary Fibrosis Emphysema

et al. 2020

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“…29 Compared with the other three clusters, cluster 2 contained more ever smokers and the FEV 1 to FVC ratio was lower; however, this cluster was not associated with a lower diffusion capacity for carbon monoxide, suggesting that these patients did not have substantial emphysema, the form of chronic obstructive pulmonary disease most commonly associated with idiopathic pulmonary fibrosis. 30 The disease trajectory in cluster 2 might reflect response to antifibrotic or immuno suppressive therapy, although patients in both studies were not receiving antifibrotic therapy at the time of recruitment, and treatment in idiopathic pulmonary fibrosis slows disease progression, rather than improves lung function. 5,6 Furthermore, it is possible that individual variation in FVC values might have resulted in unusual patterns of lung function following cluster analysis; however, this is unlikely given the large number of patients in cluster 2 and that the nature and size of the cluster were replicated in the replication cohort.…”

Section: Discussionmentioning

confidence: 99%

Forced vital capacity trajectories in patients with idiopathic pulmonary fibrosis: a secondary analysis of a multicentre, prospective, observational cohort

Fainberg

Oldham

Molyneau

et al. 2022

The Lancet Digital Health

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“…CPFE is a clinical syndrome without full recognition that is characterized by progressive worsening respiratory symptoms and markedly impaired lung diffusion function [ 2 , 3 , 26 ]. Unfortunately, limited studies have reported its prognostic risk factors [ 7 , 27 ].…”

Section: Discussionmentioning

confidence: 99%

Use of machine learning models to predict prognosis of combined pulmonary fibrosis and emphysema in a Chinese population

et al. 2022

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Background Combined pulmonary fibrosis and emphysema (CPFE) is a novel clinical entity with a poor prognosis. This study aimed to develop a clinical nomogram model to predict the 1-, 2- and 3-year mortality of patients with CPFE by using the machine learning approach, and to validate the predictive ability of the interstitial lung disease-gender-age-lung physiology (ILD-GAP) model in CPFE. Methods The data of CPFE patients from January 2015 to October 2021 who met the inclusion criteria were retrospectively collected. We utilized LASSO regression and multivariable Cox regression analysis to identify the variables associated with the prognosis of CPFE and generate a nomogram. The Harrell's C index, the calibration curve and the area under the receiver operating characteristic (ROC) curve (AUC) were used to evaluate the performance of the nomogram. Then, we performed likelihood ratio test, net reclassification improvement (NRI), integrated discrimination improvement (IDI) and decision curve analysis (DCA) to compare the performance of the nomogram with that of the ILD-GAP model. Results A total of 184 patients with CPFE were enrolled. During the follow-up, 90 patients died. After screening out, diffusing lung capacity for carbon monoxide (DLCO), right ventricular diameter (RVD), C-reactive protein (CRP), and globulin were found to be associated with the prognosis of CPFE. The nomogram was then developed by incorporating the above five variables, and it showed a good performance, with a Harrell's C index of 0.757 and an AUC of 0.800 (95% CI 0.736–0.863). Moreover, the calibration plot of the nomogram showed good concordance between the prediction probabilities and the actual observations. The nomogram also improved the discrimination ability of the ILD-GAP model compared to that of the ILD-GAP model alone, and this was substantiated by the likelihood ratio test, NRI and IDI. The significant clinical utility of the nomogram was demonstrated by DCA. Conclusion Age, DLCO, RVD, CRP and globulin were identified as being significantly associated with the prognosis of CPFE in our cohort. The nomogram incorporating the 5 variables showed good performance in predicting the mortality of CPFE. In addition, although the nomogram was superior to the ILD-GAP model in the present cohort, further validation is needed to determine the clinical utility of the nomogram.

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IPF and CPFE—The Two Different Entities or Two Different Presentations of the Same Disease?

Cited by 5 publications

References 42 publications

Prognostic Significance of Red Cell Distribution Width in Idiopathic Pulmonary Fibrosis and Combined Pulmonary Fibrosis Emphysema

Prognostic Significance of Red Cell Distribution Width in Idiopathic Pulmonary Fibrosis and Combined Pulmonary Fibrosis Emphysema

Forced vital capacity trajectories in patients with idiopathic pulmonary fibrosis: a secondary analysis of a multicentre, prospective, observational cohort

Use of machine learning models to predict prognosis of combined pulmonary fibrosis and emphysema in a Chinese population

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