The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients.70 patients newly diagnosed with IPF were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 patients was used for confirmation.Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS .3, 6-min walking distance f72% predicted and composite physiologic index .41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low diffusing coefficient of the lung for carbon monoxide. Results were confirmed in an independent cohort of patients.In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression.

In our study, muscle flap reconstruction guaranteed better early and late-term results as shown by lower rates of mortality, procedure failure and hemisternum stability. Moreover, Group 1 patients had greater postoperative VC, lower NYHA class and better quality of life. These results suggest that, in patients with multiple bone fracture, the rewiring approach does not promote physiological bone consolidation, whereas the muscle flap reconstruction can assure more physiological ventilatory dynamics.

It has been recently hypothesized that infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may lead to fibrotic sequelae in patients recovering from coronavirus disease 2019 (COVID-19). In this observational study, hospitalized patients with COVID-19 had a HRCT of the chest performed to detect the extension of fibrotic abnormalities via Hounsfield Units (HU). At follow-up, the lung density significantly improved in both lungs and in each lobe of all patients, being in the normal range (− 950 to − 700 HU). This study provides preliminary evidence that hospitalized patients with mild-to-moderate forms of COVID-19 are not at risk of developing pulmonary fibrosis.

Purpose:To correlate conventional invasive pressure indexes of pulmonary circulation with pulmonary fi rst-order arterial mean transit time (MTT) and time to peak enhancement (TTP) measured by means of three-dimensional timeresolved magnetic resonance (MR) angiography in patients with combined pulmonary fi brosis and emphysema (CPFE). Materials and Methods:The study was institutional review board approved. All subjects involved in the study provided written informed consent. Eighteen patients with CPFE were enrolled in this study. Thirteen healthy individuals matched for age and sex served as control subjects. Three-dimensional timeresolved MR angiography was performed by using a 3.0-T MR imager. Regions of interest (ROIs) were drawn manually on fi rst-order pulmonary arteries. Within the ROIs, signal intensity-versus-time curves refl ecting the fi rst pass of the contrast agent bolus in the pulmonary vessels were obtained. MTT and TTP were calculated. Pulmonary arterial pressure and pulmonary capillary wedge pressure were measured with a double-lumen, balloon-tipped catheter that was positioned in the pulmonary artery. The mean pulmonary arterial pressure (mPAP) and the pulmonary vascular resistance (PVR) were determined. Results:MTT and TTP values were prolonged signifi cantly in patients with CPFE compared with those in the control subjects ( P , .001). Mean TTP and mean MTT correlated directly with mPAP and PVR index ( P , .005). At multiple linear regression analysis, MTT was the only factor independently associated with PVR index and mPAP. Conclusion:Three-dimensional time-resolved MR angiography enables determination of pulmonary hemodynamic parameters that correlate signifi cantly with the pulmonary hemodynamic parameters obtained with invasive methods and may represent a complementary tool for evaluating pulmonary hypertension in patients with CPFE.

PET/CT allows accurate analysis of anatomical/morphological and metabolic/functional correlations of SPN, providing useful data for identifying and locating the disease, for differentiating between malignant and benign nodules and for establishing the aggressiveness and degree of vascularity of pulmonary lesions. Therefore, partly in view of the considerable reduction in time and cost of the single examinations, we believe that PET/CT will gain an increasingly dominant role in the diagnostic and therapeutic approach to lung cancer, especially in the preclinical phase.

In idiopathic pulmonary fibrosis (IPF), lung accumulation of excessive extracellular iron and macrophage haemosiderin may suggest disordered iron homeostasis leading to recurring microscopic injury and fibrosing damage.The current study population comprised 89 consistent IPF patients and 107 controls. 54 patients and 11 controls underwent bronchoalveolar lavage (BAL). Haemosiderin was assessed by Perls' stain, BAL fluid malondialdehyde (MDA) by high-performance liquid chromatography, BAL cell iron-dependent oxygen radical generation by fluorimetry and the frequency of hereditary haemochromatosis HFE gene variants by reverse dot blot hybridisation.Macrophage haemosiderin, BAL fluid MDA and BAL cell unstimulated iron-dependent oxygen radical generation were all significantly increased above controls ( p<0.05). The frequency of C282Y, S65C and H63D HFE allelic variants was markedly higher in IPF compared with controls (40.4% versus 22.4%, OR 2.35, p=0.008) and was associated with higher iron-dependent oxygen radical generation (HFE variant 107.4±56.0, HFE wild type (wt) 59.4±36.4 and controls 16.7±11.8 fluorescence units per 10 5 BAL cells; p=0.028 HFE variant versus HFE wt, p=0.006 HFE wt versus controls).The data suggest iron dysregulation associated with HFE allelic variants may play an important role in increasing susceptibility to environmental exposures, leading to recurring injury and fibrosis in IPF. @ERSpublications HFE gene variants that cause iron-related fibrosing diseases implicate lung iron dysregulation in IPF pathogenesis

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 score increased significantly, and a longitudinal increase > 7% predicted LTx-free survival significantly, with good specificity, but limited sensitivity. The correlation between radiographic and functional progression was moderately significant. HRCT progression and FVC decline predicted LTx-free survival independently and significantly, with better sensitivity, but worse specificity for a ≥ 5% decline of FVC. However, the area under the curve towards LTx-survival were only 0.61 and 0.62, respectively. Conclusions: The HRCT fibrosis visual score is a reliable and responsive tool to detect clinically meaningful disease progression. Although no individual pulmonary function test closely reflects radiographic progression, a longitudinal FVC decline improves sensitivity in the detection of clinically significant disease progression. However, the accuracy of these methods remains limited, and better prognostication models need to be found.

We reported the cases of two adult male patients who were admitted to our emergency room with abdominal pain and dyspnea caused by gallstones and pulmonary embolism respectively. During the radiological investigations, as collateral findings, we found two anomalous development of the inferior vena cava. These conditions affect about 4% of population and, although asymptomatic or mildly symptomatic, are associated with thrombotic manifestations as deep vein thrombosis and pulmonary embolism. The prompt recognition of these anomalies is necessary in order to prevent the complications associated with these conditions and to set the best therapy for patients.