High-Resolution Computed Tomography (HRCT) Reflects Disease Progression in Patients with Idiopathic Pulmonary Fibrosis (IPF): Relationship with Lung Pathology (original) (raw)
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Respiratory Research
Background Progression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course. The relationship between radiographic progression and functional decline in IPF is unclear. We sought to confirm that a simple HRCT fibrosis visual score is a reliable predictor of mortality in IPF, when longitudinally followed; and to ascertain which pulmonary functional variables best reflect clinically significant radiographic progression. Methods One-hundred-twenty-three consecutive patients with IPF from 2 centers were followed for an average of 3 years. Longitudinal changes of HRCT fibrosis scores, forced vital capacity (FVC), total lung capacity and diffusing lung capacity for carbon monoxide were considered. HRCTs were scored by 2 chest radiologists. The primary outcome was lung transplant (LTx)-free survival after the follow-up HRCT. Results During the follow-up period, 43 deaths and 11 LTx occurred. On average, the HRCT fibrosis...
The European respiratory journal, 2018
We evaluated performance characteristics and estimated the minimal clinically important difference (MCID) of data-driven texture analysis (DTA), a high-resolution computed tomography (HRCT)-derived measurement of lung fibrosis, in subjects with idiopathic pulmonary fibrosis (IPF).The study population included 141 subjects with IPF from two interventional clinical trials who had both baseline and nominal 54- or 60-week follow-up HRCT. DTA scores were computed and compared with forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide, distance covered during a 6-min walk test and St George's Respiratory Questionnaire scores to assess the method's reliability, validity and responsiveness. Anchor- and distribution-based methods were used to estimate its MCID.DTA had acceptable reliability in subjects appearing stable according to anchor variables at follow-up. Correlations between the DTA score and other clinical measurements at baseline were moderate to w...
Egyptian Journal of Bronchology, 2017
Introduction Health-related quality-of-life questionnaires need to be incorporated into the evaluations of idiopathic pulmonary fibrosis (IPF) patients to assess their influence. Aim The aim of the study was to evaluate the validity of generic and specific questionnaires in assessing the severity of IPF in correlation with high-resolution computed tomography (HRCT), diffusion lung capacity for carbon monoxide (DL CO), and cardiopulmonary exercise testing (CPET). Patients and methods Forty stable IPF patients were prospectively recruited and categorized on the basis of spirometry, DL CO , HRCT, and CPET. The results were correlated with a generic International Physical Activity Questionnaire (IPAQ) and a Specific Saint George Respiratory Questionnaire (SGRQ). Results IPF patients showed restrictive pattern with impairment of diffusion capacity (forced vital capacity (FVC)= 56±14.8% and DL CO =48.5±20% of predicted value) with a total semiquantitative scoring of HRCT 16.6±8. The mean total score of the SGRQ questionnaire for all studied cases was 56.5+21 and categorical scoring of IPAQ showed that 45, 42.5, and 12.5% of patients were in moderate, severe, and mild categories, respectively. There was a negative correlation between the total score of SGRQ and VO 2 max (ml/kg/min) (maximum oxygen consumption) (r=−0.35) and VE' (l/min) (minute ventilation) (r=−0.39) on CPET, as well as with DL CO (r=−0.53), and a positive correlation with HRCT score (r=0.63). There was a highly significant correlation between IPAQ and VO 2 max (χ 2 =28), VE' (χ 2 =14.8) and desaturation percentage variables of CPET, DL CO (r=0.61), and HRCT score (r=−0.68). Conclusion Correlations between physiological parameters including DL CO and CPET, radiological parameters in the form of HRCT, and health-related quality-of-life assessment using SGRQ and IPAQ were strong and it was possible to distinguish IPF patients with severely impaired lung functions.
Jornal Brasileiro de …, 2007
Objective: To correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF). Methods: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography (HRCT) findings were quantified: total interstitial disease (TID), reticular abnormality/honeycombing, and ground-glass opacity (GGO). The functional variables were measured by spirometry, forced oscillation technique (FOT), helium dilution method, as well as the single-breath method of measuring diffusion capacity of the lung for carbon monoxide (DLCO). Results: Of the 30 patients studied, 18 were female, and 12 were male, with a mean age of 70.9 years. We found that TID and reticular abnormality and honeycombing correlated significantly (negative correlations) with the measurements of forced vital capacity (FVC), total lung capacity (TLC), DLCO, and dynamic respiratory compliance were found, as well as that GGO correlated significantly (and positively) with residual volume/TLC. The ratio of forced expiratory flow between 25 and 75% of FVC to FVC (FEF 25-75% /FVC) correlated positively with TID, reticular abnormality/honeycombing, and GGO. Conclusion: In IPF patients, the measurements of volume, diffusion, and dynamic compliance are the physiological variables which best reflect the extent of the interstitial disease on HRCT scans.
High-Resolution Computed Tomography in Idiopathic Pulmonary Fibrosis
American Journal of Respiratory and Critical Care Medicine, 2005
Rationale: High-resolution computed tomography (HRCT) is an integral aspect of the evaluation of patients with suspected idiopathic pulmonary fibrosis (IPF). However, few studies have evaluated its use in a large cohort. Objectives: To describe HRCT features in patients with mild to moderate IPF, compare diagnostic evaluations by a radiology core (three thoracic radiologists) with those by studysite radiologists, correlate baseline clinical and physiologic variables with HRCT findings, and evaluate their association with mortality. Methods: We assessed HRCT scans from patients with IPF (n ϭ 315) enrolled in a randomized controlled study evaluating IFN-␥1b.
Predicting Outcomes in Idiopathic Pulmonary Fibrosis Using Automated Computed Tomographic Analysis
American journal of respiratory and critical care medicine, 2018
Quantitative computed tomographic (CT) measures of baseline disease severity might identify patients with idiopathic pulmonary fibrosis (IPF) with an increased mortality risk. We evaluated whether quantitative CT variables could act as a cohort enrichment tool in future IPF drug trials. To determine whether computer-derived CT measures, specifically measures of pulmonary vessel-related structures (VRSs), can better predict functional decline and survival in IPF and reduce requisite sample sizes in drug trial populations. Patients with IPF undergoing volumetric noncontrast CT imaging at the Royal Brompton Hospital, London, and St. Antonius Hospital, Utrecht, were examined to identify pulmonary function measures (including FVC) and visual and computer-derived (CALIPER [Computer-Aided Lung Informatics for Pathology Evaluation and Rating] software) CT features predictive of mortality and FVC decline. The discovery cohort comprised 247 consecutive patients, with validation of results con...
Internal Medicine, 2018
Objective An image analysis of high-resolution computed tomography (HRCT) can provide objective quantitation of the disease status in idiopathic pulmonary fibrosis (IPF). However, to our knowledge, no reports have investigated the utility of the normal lung volume for evaluating mortality from IPF. This study aimed to evaluate the relationship between the normally attenuated lung volume on HRCT as a percentage of whole-lung volume (NL%) and IPF mortality. Methods The NL% was determined by HRCT (between-950 and-701 Hounsfield units) using a density mask technique and volumetric software. The NL%, visual assessments of the normal lung by two radiologists, pulmonary function variables, and the gender, age, and physiology (GAP) index were retrospectively evaluated for 175 patients with IPF. Uni-and multivariate Cox proportional hazards analyses and C statistics for mortality were performed. Results The univariate Cox proportional hazards analysis identified the NL% as a prognostic factor [hazard ratio, 0.949; 95% confidence interval (CI), 0.936-0.964; p<0.0001]. In the multivariate analysis, the NL% was a prognostic factor, but the radiologists' visual assessment scores of normal lung were not. The C index increased when the NL% was included in the models of the pulmonary function variables. Furthermore, the C index for a combined model of GAP stage and categorized NL% (0.758; 95% CI, 0.751-0.762) was higher than for the model with the GAP stage alone (0.689; 95% CI, 0.672-0.709). Conclusion The NL% was a prognostic factor in our study population. Quantification of the normal lung using our method may help improve the IPF staging systems.
American journal of respiratory and critical care medicine, 2018
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with great variability in disease severity and rate of progression. The need for a reliable, sensitive, and objective biomarker to track disease progression and response to therapy remains a great challenge in IPF clinical trials. Over the past decade, quantitative computed tomography (QCT) has emerged as an area of intensive research to address this need. We have gathered a group of pulmonologists, radiologists and scientists with expertise in this area to define the current status and future promise of this imaging technique in the evaluation and management of IPF. In this Pulmonary Perspective, we review the development and validation of six computer-based QCT methods and offer insight into the optimal use of an imaging-based biomarker as a tool for prognostication, prediction of response to therapy, and potential surrogate endpoint in future therapeutic trials.
Thorax, 2020
AimsPatients with idiopathic pulmonary fibrosis (IPF) receiving antifibrotic medication and patients with non-IPF fibrosing lung disease often demonstrate rates of annualised forced vital capacity (FVC) decline within the range of measurement variation (5.0%–9.9%). We examined whether change in visual CT variables could help confirm whether marginal FVC declines represented genuine clinical deterioration rather than measurement noise.MethodsIn two IPF cohorts (cohort 1: n=103, cohort 2: n=108), separate pairs of radiologists scored paired volumetric CTs (acquired between 6 and 24 months from baseline). Change in interstitial lung disease, honeycombing, reticulation, ground-glass opacity extents and traction bronchiectasis severity was evaluated using a 5-point scale, with mortality prediction analysed using univariable and multivariable Cox regression analyses. Both IPF populations were then combined to determine whether change in CT variables could predict mortality in patients wit...
High-resolution computed tomography in the diagnosis and follow-up of idiopathic pulmonary fibrosis
La radiologia medica, 2010
Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease and is associated with a fatal prognosis. Familiarity with the typical appearances of IPF on high-resolution computed tomography (HRCT) is important, as in the appropriate clinical setting, it is often sufficient for establishing a confident diagnosis of IPF without the need for surgical biopsy. Moreover, HRCT can provide important prognostic information in IPF. This is noteworthy, as the course of IPF is variable, and many patients develop complications leading to respiratory failure and death. The purpose of this paper is to review the progress made towards a better understanding of the HRCT patterns of IPF.