Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis

This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung. The CT scores were evaluated against functional indices forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide (DLCO), transfer coefficient of the lung for carbon monoxide (KCO), composite physiologic index (CPI)) and mortality.The presence and extent of emphysema had no impact on survival. Results were maintained following correction for age, gender, smoking status and baseline severity usingDLCO, and combined visual emphysema and ILD extent. Visual emphysema quantitation indicated that relative preservation of lung volumes (FVC) resulted from tractionally dilated airways within fibrotic lung, ventilating areas of admixed emphysema (p<0.0001), with no independent effect on FVC from isolated emphysema. Conversely, only isolated emphysema (p<0.0001) reduced gas transfer (DLCO).There is no prognostic impact of emphysema in IPF, beyond that explained by the additive extents of both fibrosis and emphysema. With respect to the location of pulmonary fibrosis, emphysema distribution determines the functional effects of emphysema.

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Mortality prediction in idiopathic pulmonary fibrosis: evaluation of computer-based CT analysis with conventional severity measures

European Respiratory Journal ◽

10.1183/13993003.01011-2016 ◽

2016 ◽

Vol 49 (1) ◽

pp. 1601011 ◽

Cited By ~ 97

Author(s):

Joseph Jacob ◽

Brian J. Bartholmai ◽

Srinivasan Rajagopalan ◽

Maria Kokosi ◽

Arjun Nair ◽

...

Keyword(s):

Carbon Monoxide ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Function ◽

Forced Expiratory Volume ◽

Mortality Prediction ◽

Pulmonary Vessel ◽

Staging Systems ◽

Ct Analysis ◽

Computer Based

Computer-based computed tomography (CT) analysis can provide objective quantitation of disease in idiopathic pulmonary fibrosis (IPF). A computer algorithm, CALIPER, was compared with conventional CT and pulmonary function measures of disease severity for mortality prediction.CT and pulmonary function variables (forced expiratory volume in 1 s, forced vital capacity, diffusion capacity of the lung for carbon monoxide, transfer coefficient of the lung for carbon monoxide and composite physiologic index (CPI)) of 283 consecutive patients with a multidisciplinary diagnosis of IPF were evaluated against mortality. Visual and CALIPER CT features included total extent of interstitial lung disease, honeycombing, reticular pattern, ground glass opacities and emphysema. In addition, CALIPER scored pulmonary vessel volume (PVV) while traction bronchiectasis and consolidation were only scored visually. A combination of mortality predictors was compared with the Gender, Age, Physiology model.On univariate analyses, all visual and CALIPER-derived interstitial features and functional indices were predictive of mortality to a 0.01 level of significance. On multivariate analysis, visual CT parameters were discarded. Independent predictors of mortality were CPI (hazard ratio (95% CI) 1.05 (1.02–1.07), p<0.001) and two CALIPER parameters: PVV (1.23 (1.08–1.40), p=0.001) and honeycombing (1.18 (1.06–1.32), p=0.002). A three-group staging system derived from this model was powerfully predictive of mortality (2.23 (1.85–2.69), p<0.0001).CALIPER-derived parameters, in particular PVV, are more accurate prognostically than traditional visual CT scores. Quantitative tools such as CALIPER have the potential to improve staging systems in IPF.

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Prognostic impact of malignant diseases in idiopathic pulmonary fibrosis

Scientific Reports ◽

10.1038/s41598-020-75276-2 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Hong Yeul Lee ◽

Jaeyoung Cho ◽

Nakwon Kwak ◽

Jinwoo Lee ◽

Young Sik Park ◽

...

Keyword(s):

Lung Cancer ◽

Carbon Monoxide ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Medical Records ◽

Vital Capacity ◽

Prognostic Impact ◽

Malignant Diseases ◽

Diffusion Capacity ◽

And Diffusion

Abstract No studies on idiopathic pulmonary fibrosis (IPF) have investigated the prognostic impact of extrapulmonary cancers in patients with IPF. We aimed to determine the prognostic impact of malignancies in patients with IPF. We retrospectively reviewed the medical records of patients diagnosed with IPF between 2001 and 2015. Patients were divided into three groups: IPF without cancer (n = 440), IPF with lung cancer (n = 69), and IPF with extrapulmonary cancer (n = 70). Of the 579 patients with IPF, 139 (24%) had cancer; the three most common types were lung (11.9%), gastric (2.4%), and colorectal (1.9%). Survival was significantly worse in patients with lung cancer than in those without cancer (hazard ratio [HR] = 1.83, 95% confidence interval [CI], 1.35–2.48) or those with extrapulmonary cancer (HR = 1.70, 95% CI, 1.14–2.54). The rate of hospitalisation for cancer-related complications was significantly higher in IPF patients with lung cancer than in those with extrapulmonary cancer. The annual rates of decline in percent predicted forced vital capacity and diffusion capacity for carbon monoxide did not differ among the groups. Physicians should pay attention to the development and progression of cancer and its prognostic impact in patients with IPF.

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Quantitative CT analysis using functional imaging is superior in describing disease progression in idiopathic pulmonary fibrosis compared to forced vital capacity

Respiratory Research ◽

10.1186/s12931-018-0918-5 ◽

2018 ◽

Vol 19 (1) ◽

Cited By ~ 8

Author(s):

J. Clukers ◽

M. Lanclus ◽

B. Mignot ◽

C. Van Holsbeke ◽

J. Roseman ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Disease Progression ◽

Functional Imaging ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Quantitative Ct ◽

Ct Analysis ◽

Quantitative Ct Analysis

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Quantitative CT analysis of honeycombing area in idiopathic pulmonary fibrosis: Correlations with pulmonary function tests

European Journal of Radiology ◽

10.1016/j.ejrad.2015.11.011 ◽

2016 ◽

Vol 85 (1) ◽

pp. 125-130 ◽

Cited By ~ 15

Author(s):

Hiroaki Nakagawa ◽

Yukihiro Nagatani ◽

Masashi Takahashi ◽

Emiko Ogawa ◽

Nguyen Van Tho ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Function ◽

Pulmonary Function Tests ◽

Quantitative Ct ◽

Function Tests ◽

Ct Analysis ◽

Quantitative Ct Analysis

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Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis

Scientific Reports ◽

10.1038/s41598-018-32478-z ◽

2018 ◽

Vol 8 (1) ◽

Cited By ~ 17

Author(s):

Yuzo Suzuki ◽

Katsuhiro Yoshimura ◽

Yasunori Enomoto ◽

Hideki Yasui ◽

Hironao Hozumi ◽

...

Keyword(s):

Body Composition ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Prognostic Impact ◽

Pleuroparenchymal Fibroelastosis ◽

Composition Changes

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Prognostic impact of the ratio of the main pulmonary artery to that of the aorta on chest computed tomography in patients with idiopathic pulmonary fibrosis

BMC Pulmonary Medicine ◽

10.1186/s12890-019-0843-5 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Ji Soo Choi ◽

Sang Hoon Lee ◽

Ah. Young Leem ◽

Joo Han Song ◽

Kyung Soo Chung ◽

...

Keyword(s):

Computed Tomography ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Artery ◽

Main Pulmonary Artery ◽

Prognostic Impact ◽

Chest Computed Tomography

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Determinants of bronchoalveolar lavage cellularity in idiopathic pulmonary fibrosis

Journal of Applied Physiology ◽

10.1152/jappl.1991.71.5.1688 ◽

1991 ◽

Vol 71 (5) ◽

pp. 1688-1693 ◽

Cited By ~ 25

Author(s):

D. A. Schwartz ◽

R. A. Helmers ◽

C. S. Dayton ◽

R. K. Merchant ◽

G. W. Hunninghake

Keyword(s):

Multivariate Analysis ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Cigarette Smoking ◽

Bronchoalveolar Lavage ◽

Alveolar Macrophages ◽

Smoking Status ◽

Physiological Measures ◽

Multivariate Models ◽

Normal Volunteers

To investigate factors that determine bronchoalveolar lavage (BAL) cellularity in patients with idiopathic pulmonary fibrosis (IPF), we compared BAL cells in patients with IPF (n = 83) to both nonsmoking (n = 111) and smoking (n = 19) normal volunteers. Patients with IPF had higher concentrations of BAL total cells and alveolar macrophages than nonsmoking volunteers and more BAL neutrophils and eosinophils than normal volunteers regardless of smoking status. Among patients with IPF, the numbers of alveolar macrophages, neutrophils, or eosinophils were strongly associated with either smoking status or pack-years of cigarette smoking. In fact, after accounting for cigarette smoking, using multivariate analysis, the only additional factors that were found to be associated with BAL cellularity were age (macrophages and eosinophils) and the percent predicted forced expired volume in 1 s (neutrophils). Additional multivariate models failed to identify a significant relationship between BAL cellularity and either the type of immunosuppressive therapy or other physiological measures of lung function. We conclude that cigarette smoking strongly influences BAL cellularity in patients with IPF. These findings suggest that cigarette smoking may have a role in the pathogenesis of IPF or may adversely affect the prognosis in patients with IPF.

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