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

Suzuki, Masaki; Kawata, Naoko; Abe, Mitsuhiro; Yokota, Hajime; Anazawa, Rie; Matsuura, Yukiko; Ikari, Jun; Matsuoka, Shin; Tsushima, Kenji

doi:10.1371/journal.pone.0239066

“…The prognostic impact of the proportion of abnormal lesions has also been studied in patients with CPFE. Suzuki et al [ 51 ] showed that an increased proportion of abnormal area (i.e., sum of low attenuation area [pixels <–950 Hounsfield units, representing emphysema] and high attenuation area [pixels >–700 Hounsfield units, representing interstitial fibrosis]) in chest CT was significantly associated with frequent hospitalization in patients with CPFE. Nemoto et al [ 82 ] analyzed fibrotic area alone in patients with CPFE, which revealed that patients with a higher proportion of fibrosis had higher mortality, more frequent exacerbations, worse lung function trajectories, and more rapid CPFE progression.…”

Section: Prognosismentioning

confidence: 99%

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

Choi

¹

,

Song

²

,

Rhee

³

2022

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Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension. The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.

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“…These variables are comprehensive and easily available, thus facilitating decision-making by physicians. Third, quantitative indicators of fibrosis or emphysema were not included in the study, but the more objective indicators, lung function parameters, were included because of the risk of collinearity [ 48 , 49 ]. Fourth, the diagnosis of PH in the study was based on echocardiography instead of right heart catheterization (RHC).…”

Section: Discussionmentioning

confidence: 99%

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

Liu

¹

,

Sun

²

,

Wang

³

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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“…Emphysematous lesions of CPFE can be presented as centrilobular, paraseptal or mixed lesion of both type. Among these manifestations, there were discrepancies between studies on majority type of emphysema; centrilobular [50][51][52] , paraseptal 9,[53][54][55] or mixed type 10,56 were reported to be most common type of emphysema in different studies. Regarding fibrotic area of CPFE, usual interstitial pneumonia has been shown to be the most common histological morphology in previous studies 9,57 .…”

Section: Radiologic Featuresmentioning

confidence: 94%

“…The prognostic impact of the proportion of abnormal lesions has also been studied in patients with CPFE. Suzuki et al showed that an increased proportion of abnormal area (i.e., sum of low attenuation area [pixels < -950 Hounsfield units, representing emphysema] and high attenuation area [pixels > -700 Hounsfield units, representing interstitial fibrosis]) in chest CT was significantly associated with frequent hospitalization in patients with CPFE 51 .…”

Section: Prognosis Of Cpfementioning

confidence: 99%

Chronic obstructive pulmonary disease combined with interstitial lung disease

Choi¹,

Song²,

Rhee³

2022

Tuberc Respir Dis

3

0

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Although chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) have distinct clinical features, both diseases may coexist in a patient because they share similar risk factors such as smoking, male sex, and old age. Patients with both emphysema in upper lung fields and diffuse ILD are diagnosed with combined pulmonary fibrosis and emphysema (CPFE), which causes substantial clinical deterioration. Patients with CPFE have higher mortality compared with patients who have COPD alone, but results have been inconclusive compared with patients who have idiopathic pulmonary fibrosis (IPF). Poor prognostic factors for CPFE include exacerbation, lung cancer, and pulmonary hypertension.The presence of interstitial lung abnormalities, which may be an early or mild form of ILD, is notable among patients with COPD, and is associated with poor prognosis. Various theories have been proposed regarding the pathophysiology of CPFE. Biomarker analyses have implied that this pathophysiology may be more closely associated with IPF development, rather than COPD or emphysema. Patients with CPFE should be advised to quit smoking and undergo routine lung function tests, and pulmonary rehabilitation may be helpful. Various pharmacologic agents and surgical approaches may be beneficial in patients with CPFE, but further studies are needed.Additional comparisons of biomarkers between groups revealed four clear categories: biomarkers elevated in emphysema alone (interleukin [IL]-6, CXCL10, CCL2, IL-10, and interferon-γ); biomarkers reduced in emphysema alone (IL-17A, IL-13, matrix metalloproteinase [MMP] 1, MMP13, MMP2, CCL24, and lysyl oxidase); biomarkers that were similar among emphysema, IPF, and CPFE, but different from the healthy control group (sCD14, CCL5, tumor necrosis factor [TNF] RII, SP-D, ST2, IL-1β, IL-4, IL-5, CXCL8, and TNFα); and biomarkers with a mixed pattern that did not fit any other categories. These results suggest that the levels of inflammatory proteins are generally comparable between IPF and CPFE, but distinct features are present in emphysema. These results were also consistent with the findings of previous studies [38][39][40][41] . Therefore, the immunological pathogenesis of CPFE may be more closely associated with IPF development.Klebs von den Lungen-6 (KL-6) is a high-molecular-weight glycoprotein that is strongly expressed on type II alveolar pneumocytes and bronchiolar epithelial cells. It is an important marker of ILD and is associated with disease severity, progression, and survival; notably, it promotes pulmonary fibroblast migration and proliferation [42][43][44][45] . Because of this association with ILD, some studies have investigated relationships with fibrotic changes in patients with COPD. In asthma-COPD overlap, patients with a history of smoking and older age have more frequent ILA 46 . These patients with asthma-COPD overlap and ILA showed higher levels of KL-6, compared with patients with asthma-COPD overlap who lacked ILA.The clinical significance of KL-6 has also bee...

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

Cited by 8 publications

References 58 publications

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease

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

Chronic obstructive pulmonary disease combined with interstitial lung disease

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