Assessment of emphysema-modifying therapy is difficult, but newer outcome measures offer advantages over traditional methods. The EXAcerbations and Computed Tomography scan as Lung End-points (EXACTLE) trial explored the use of computed tomography (CT) densitometry and exacerbations for the assessment of the therapeutic effect of augmentation therapy in subjects with a 1 -antitrypsin (a 1 -AT) deficiency.In total, 77 subjects (protease inhibitor type Z) were randomised to weekly infusions of 60 mg?kg -1 human a 1 -AT (Prolastin1) or placebo for 2-2.5 yrs. The primary end-point was change in CT lung density, and an exploratory approach was adopted to identify optimal methodology, including two methods of adjustment for lung volume variability and two statistical approaches.Other end-points were exacerbations, health status and physiological indices. CT was more sensitive than other measures of emphysema progression, and the changes in CT and forced expiratory volume in 1 s were correlated. All methods of densitometric analysis concordantly showed a trend suggestive of treatment benefit (p-values for Prolastin1 versus placebo ranged 0.049-0.084). Exacerbation frequency was unaltered by treatment, but a reduction in exacerbation severity was observed.In patients with a 1 -AT deficiency, CT is a more sensitive outcome measure of emphysemamodifying therapy than physiology and health status, and demonstrates a trend of treatment benefit from a 1 -AT augmentation.

Abstract-We aim at improving quantitative measures of emphysema in computed tomography (CT) images of the lungs. Current standard measures, such as the relative area of emphysema (RA), rely on a single intensity threshold on individual pixels, thus ignoring any interrelations between pixels. Texture analysis allows for a much richer representation that also takes the local structure around pixels into account. This paper presents a texture classification-based system for emphysema quantification in CT images. Measures of emphysema severity are obtained by fusing pixel posterior probabilities output by a classifier. Local binary patterns (LBP) are used as texture features, and joint LBP and intensity histograms are used for characterizing regions of interest (ROIs). Classification is then performed using a nearest neighbor classifier with a histogram dissimilarity measure as distance. A 95.2% classification accuracy was achieved on a set of 168 manually annotated ROIs, comprising the three classes: normal tissue, centrilobular emphysema, and paraseptal emphysema. The measured emphysema severity was in good agreement with a pulmonary function test (PFT) achieving correlation coefficients of up to = 0 79 in 39 subjects. The results were compared to RA and to a Gaussian filter bank, and the texture-based measures correlated significantly better with PFT than did RA.

The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family caregivers. Both patients and family caregivers face an altered life as the disease progresses. The aim of our study was to increase knowledge of life with IPF for patients and family caregivers.This study had a qualitative descriptive design using in-depth dyadic interviews with IPF patients (n=25) and family caregivers (n=24). We used the five-step analysis from the framework method and analysed the data on three levels: the patient, the family caregivers and couple level.The following six themes emerged as the main results: information and disclosure, reactional dyssynchrony, perpetual vigilance, emotional ambivalence, gradual and tacit role shift, and adapted coping strategies.Our findings suggest that IPF patients need information at the time of diagnosis, but some issues should be paced as the disease progresses. A palliation plan was demanded by patients and their caregivers. Further efforts are required to provide palliative care to IPF patients starting at the time of diagnosis. @ERSpublications IPF patients and caregivers need information, but full disclosure should be gradual and palliation initiated early

The purposes of this study were: to describe chest CT findings in normal non-smoking controls and cigarette smokers with and without COPD; to compare the prevalence of CT abnormalities with severity of COPD; and to evaluate concordance between visual and quantitative chest CT (QCT) scoring Methods Volumetric inspiratory and expiratory CT scans of 294 subjects, including normal non-smokers, smokers without COPD, and smokers with GOLD Stage I-IV COPD, were scored at a multi-reader workshop using a standardized worksheet. There were fifty-eight observers (33 pulmonologists, 25 radiologists); each scan was scored by 9–11 observers. Interobserver agreement was calculated using kappa statistic. Median score of visual observations was compared with QCT measurements. Results Interobserver agreement was moderate for the presence or absence of emphysema and for the presence of panlobular emphysema; fair for the presence of centrilobular, paraseptal, and bullous emphysema subtypes and for the presence of bronchial wall thickening; and poor for gas trapping, centrilobular nodularity, mosaic attenuation, and bronchial dilation. Agreement was similar for radiologists and pulmonologists. The prevalence on CT readings of most abnormalities (e.g. emphysema, bronchial wall thickening, mosaic attenuation, expiratory gas trapping) increased significantly with greater COPD severity, while the prevalence of centrilobular nodularity decreased. Concordances between visual scoring and quantitative scoring of emphysema, gas trapping and airway wall thickening were 75%, 87% and 65%, respectively. Conclusions Despite substantial inter-observer variation, visual assessment of chest CT scans in cigarette smokers provides information regarding lung disease severity; visual scoring may be complementary to quantitative evaluation.