Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases

Kumar, Anupam; Cherian, Sujith V.; Vassallo, Robert; Yi, Eunhee S.; Ryu, Jay H.

doi:10.1016/j.chest.2017.11.023

Cited by 84 publications

(62 citation statements)

References 110 publications

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“…Although Wiggins et al [4] rst reported combined cryptogenic brosing alveolitis and emphysema in eight cigarette smokers in the 1980s, with a unique presentation of severe breathlessness and low gas transfer without apparent air ow obstruction; CPFE remained poorly recognised until the 2000s [5]. In 2010, many studies conducted on CPFE in IPF recognised a poor prognosis due to the high prevalence of pulmonary hypertension and lung cancer [2,5,29]; the current study also showed that IPF is associated with poor outcome. Alsumrain et al reported a similar proportion of IPF to that in our study in their CPFE cohort [10], and the remaining patients were either unclassi able or diagnosed with secondary ILD using clinicoradiological characteristics.…”

Section: Discussionmentioning

confidence: 56%

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

Nemoto

Nei

Bartholmai

et al. 2020

Preprint

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Background: Combined pulmonary fibrosis and emphysema (CPFE) is a heterogeneous clinico-radiological syndrome without a consensus definition. There are limited data on the relation between the amount of parenchymal fibrosis and prognosis. In this study, we assessed the prognostic implications of the extent of fibrosis assessed by an automated quantitative computed tomography (CT) technique and the radiological and functional change over time in patients with a broad spectrum of fibrotic interstitial lung diseases (ILDs) encountered in a real-world setting.Methods: We conducted a single-centre, retrospective study of 228 consecutive patients with CPFE, encountered from 2007 to 2015 at Kameda Medical Center, Chiba, Japan. We investigated the prognostic value of automated CT fibrosis quantification and the subsequent course of CPFE. Results: Among 228 patients with CPFE, 89 had fibrosis affecting <5% of their lungs, 54 had 5 to <10% fibrosis, and 85 had ≥10% fibrosis at the time of diagnosis. Lower volume of fibrosis correlated with lower rates of mortality and acute exacerbation (p<0.001). In particular, among those with <5% fibrosis, only 4.5% died and none experienced acute exacerbation during follow-up, whereas 57.6% and 29.4% of those with ≥10% fibrosis experienced death and acute exacerbation, respectively. The Cox proportional hazard model for CPFE progression (defined by composite criteria of death, acute exacerbation, and decline in forced vital capacity or diffusing capacity) showed fibrosis proportion was a risk factor independent of age, sex, smoking pack-years, the Charlson Comorbidity Index, lung cancer, connective tissue disease, and idiopathic pulmonary fibrosis.Conclusions: Less severe (<5%) fibrosis at baseline was associated with disease stability and better prognosis compared to more severe fibrosis in CPFE occurring with fibrotic ILDs. Further studies including a validation cohort will be needed.Trial Registration: retrospectively registered

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Section: Discussionmentioning

confidence: 56%

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

Nemoto

Nei

Bartholmai

et al. 2020

Preprint

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“…In the “ligand-dependent nuclear factor” category, NR3C1 (gene encoding glucocorticoid receptor) was predicted to be significantly inhibited (Z-score −2.235) (Figure 3). This may be reflecting the fact that corticosteroids are considered as an effective treatment in some cases of DIP (10). In contrast, PPARA was predicted to be significantly activated (Z-score 2.159).…”

Section: Resultsmentioning

confidence: 99%

Transcriptome Analysis of Desquamative Interstitial Pneumonia (DIP)

Saito

Kolls

Liu

et al. 2019

Preprint

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Desquamative interstitial pneumonia (DIP) is a rare diffuse parenchymal lung disease of unclear etiology. A recent study showed that mice overexpressing granulocyte macrophage colony-stimulating factor (GM-CSF) in lungs develop DIP-like disease, suggesting that pulmonary GM-CSF may be involved in the pathogenesis of DIP. To determine if GM-CSF is involved in human DIP lungs, we performed transcriptome analysis on human DIP lung tissue. We also extended transcriptome analysis to respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), which has been thought to be in the same spectrum of disease. The analysis revealed that DIP has a distinct transcriptome profile compared to both RB-ILD and non-diseased lung controls. It also suggested that GM-CSF was a key upstream regulator in the DIP transcriptome and that the GM-CSF signaling pathway was highly activated in DIP tissue. Further bioinformatics analysis using xCell, a novel computational method that assesses enrichments of individual cell types based on gene expression, suggested that DIP is enriched for gene signatures of macrophages and other immune cells such as dendritic cells and B cells. In conclusion, our analysis shows that DIP is characterized by a GM-CSF signature, and thus GM-CSF is likely to be involved in the pathogenesis of DIP. Our analysis also suggests that immune cells other than alveolar macrophages, such as B cells, may also be involved in the pathogenesis of DIP.

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“…In the absence of consensus criteria for CPFE, we avoided using the minimum brosis or emphysema criteria and analysed the entire patient cohort with any amount of bilateral lung brosis and emphysema to avoid selection bias. The exclusion criteria were: (1) no mention of emphysema in the chest CT report; (2) diagnosis of other speci c types of ILD, following the prior publication by Cottin V, et al, such as druginduced ILD, pneumoconiosis, hypersensitivity pneumonitis, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, or eosinophilic pneumonias [5,7]; (3) no history of cigarette smoking (to avoid other forms of cystic lung diseases) [2]; and (4) no follow-up chest CT or PFTs >1 year after the initial evaluation, unless the patient had experienced acute exacerbation or had died.…”

Section: Study Design and Participantsmentioning

confidence: 99%

“…Combined pulmonary brosis and emphysema (CPFE) is a heterogeneous clinico-radiological syndrome, comprising emphysema in the upper lung and brosis in the lower lung. CPFE is strongly associated with cigarette smoking thus, considered a smoking-related interstitial lung disease (ILD) [1,2]. Natural disease history differs between patients with CPFE and those with either pulmonary brosis or emphysema alone.…”

Section: Introductionmentioning

confidence: 99%

“…Although initial reports of CPFE cases were described in patients with IPF [4], recent studies included brotic ILDs with computed tomography (CT) ndings inconsistent with a usual interstitial pneumonia (UIP) pattern [5]. In the absence of a consensus de nition for CPFE, it seems important to analyse all patients with co-existent emphysema and brotic ILDs (rather than just IPF/UIP) seen on high resolution CT to clarify the prognostic implications of CPFE in the broader population [2].…”

Section: Introductionmentioning

confidence: 99%

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Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

Nemoto

Nei

Bartholmai

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

Background: Combined pulmonary fibrosis and emphysema (CPFE) is a heterogeneous clinico-radiological syndrome without a consensus definition. There are limited data on the relation between the amount of parenchymal fibrosis and prognosis. In this study, we assessed the prognostic implications of the extent of fibrosis assessed by an automated quantitative computed tomography (CT) technique and the radiological and functional change over time in patients with a broad spectrum of fibrotic interstitial lung diseases (ILDs) encountered in a real-world setting.Methods: We conducted a single-centre, retrospective study of 228 consecutive patients with CPFE, encountered from 2007 to 2015 at Kameda Medical Center, Chiba, Japan. We investigated the prognostic value of automated CT fibrosis quantification and the subsequent course of CPFE. Results: Among 228 patients with CPFE, 89 had fibrosis affecting <5% of their lungs, 54 had 5 to <10% fibrosis, and 85 had ≥10% fibrosis at the time of diagnosis. Lower volume of fibrosis correlated with lower rates of mortality and acute exacerbation (p<0.001). In particular, among those with <5% fibrosis, only 4.5% died and none experienced acute exacerbation during follow-up, whereas 57.6% and 29.4% of those with ≥10% fibrosis experienced death and acute exacerbation, respectively. Although, the ≥10% fibrosis group had the poorest overall survival as well as the highest incidence of acute exacerbation, the incidence of decline in pulmonary function tests, change per year in total lung volume, and progression of fibrosis on chest CT was highest in the 5 to <10% fibrosis group. The Cox proportional hazard model for CPFE progression (defined by composite criteria of death, acute exacerbation, and decline in forced vital capacity or diffusing capacity) showed fibrosis proportion was a risk factor independent of age, sex, smoking pack-years, the Charlson Comorbidity Index, lung cancer, connective tissue disease, and idiopathic pulmonary fibrosis.Conclusions: Less severe (<5%) fibrosis at baseline was associated with disease stability and better prognosis compared to more severe fibrosis in CPFE occurring with fibrotic ILDs. Further studies including a validation cohort will be needed.Trial Registration: retrospectively registered

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Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases

Cited by 84 publications

References 110 publications

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

Transcriptome Analysis of Desquamative Interstitial Pneumonia (DIP)

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting-A single-centre, retrospective study

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