Acute exacerbation of idiopathic pulmonary fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of idiopathic pulmonary fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of idiopathic pulmonary fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in idiopathic pulmonary fibrosis and a revised definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis.

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.

Spiral CT has greater accuracy and specificity than V-P scanning in patients with an unresolved diagnosis and may be useful as the primary screening technique for PE.

High-resolution computed tomography (HRCT) is widely used to assess pulmonary parenchymal disease in adults. The authors used ultrafast CT with 3-mm collimation and a 100-msec scan time to obtain HRCT scans in 36 children (mean age, 49.4 months). Clinical diagnoses included normal lungs (n = 6), cystic fibrosis (n = 12), obliterative bronchiolitis (n = 6), idiopathic pulmonary hemosiderosis (n = 2), and other lung diseases (n = 10). The HRCT scans and chest radiographs were reviewed separately in blinded fashion. Pulmonary parenchymal abnormalities were categorized into interstitial, airspace, and airway processes. Nine patients with normal chest radiographs had abnormal HRCT scans. In five other patients, the extent of abnormal lung parenchyma was considerably greater on HRCT scans than on chest radiographs. HRCT scans allowed accurate characterization of the type of lung process in 24 of 30 patients when compared with clinical or biopsy findings. Early HRCT findings in cystic fibrosis included lobular air trapping, bronchial wall thickening, and centrilobular nodules. The use of cine CT with high-resolution techniques is feasible in children too young or too sick to hold their breath. HRCT may enable early detection and characterization of pulmonary disease and depiction of the extent of lung abnormality.

B Br ro on nc ch hi io ol li it ti is s o ob bl li it te er ra an ns s: : t th he e l lo on ne e m ma an ni if fe es st ta at ti io on n o of f r rh he eu um ma at to oi id d a ar rt th hr ri it ti is s? ? This case is unusual since it appeared in a man and is the first and, so far, the only manifestation of rheumatoid arthritis. Prior studies indicate that the bronchiolitis obliterans of rheumatoid arthritis occurs primarily in women and only in ongoing cases.