Lung microhaemorrhage drives oxidative/inflammatory damage in α-antitrypsin deficiency.

Background: Animal models using intratracheal instillation show that elastase, unopposed by α-antitrypsin (AAT), causes alveolar damage and haemorrhage associated with emphysematous changes. The aim of the present study was to characterise any relationship between alveolar haemorrhage and human AAT deficiency (AATD) using bronchoalveolar lavage (BAL) and lung explant samples from AATD subjects.

Methods: BAL samples (17 patients, 15 controls) were evaluated for free haem (iron protoporphyrin IX) and total iron concentrations.

Analysis of single nucleotide polymorphisms in chronic beryllium disease.

Sarcoidosis and chronic beryllium disease (CBD) are phenocopies, however the latter one has a clear trigger factor that is beryllium exposure. This study analyses single nucleotide polymorphisms (SNPs) in a large cohort for beryllium-exposed persons. SNPs were chosen for their relevance in sarcoidosis.

An Efficient Algorithm for Identifying Mutated Subnetworks Associated with Survival in Cancer.

Protein-protein interaction (PPI) network models interconnections between protein-encoding genes. A group of proteins that perform similar functions are often connected to each other in the PPI network. The corresponding genes form pathways or functional modules.

Bronchoalveolar lavage (BAL) cells in idiopathic pulmonary fibrosis express a complex pro-inflammatory, pro-repair, angiogenic activation pattern, likely associated with macrophage iron accumulation.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown cause characterized by alveolar epithelial damage, patchy interstitial fibrosis and diffuse microvascular abnormalities. In IPF, alveolar clustering of iron-laden alveolar macrophages-a common sign of microhemorrhage, has been associated with vascular abnormalities and worsening of pulmonary hypertension. As iron-dependent ROS generation has been shown to induce unrestrained macrophage activation in disease models of vascular damage, we explored alveolar macrophage activation phenotype in IPF patients (n = 16) and healthy controls (CTR, n = 7) by RNA sequencing of bronchoalveolar lavage (BAL) cells.

Structured multidisciplinary discussion of HRCT scans for IPF/UIP diagnosis may result in indefinite outcomes.

Recent ATS/ERS/JRS/ALAT guidelines for the diagnosis and management of Idiopathic Pulmonary Fibrosis (IPF) have defined key features and specific high-resolution computerized tomography (HRCT) patterns for the diagnosis of UIP. The aim is the sorting of patients with suspected IPF into three subgroups, confident, possible or inconsistent with UIP patterns, after a multidisciplinary discussion (MDD). Specialists in respiratory diseases, radiologists and pathologists should reach IPF diagnosis based on either patients' clinical, radiological and laboratory data, either submitting patients to surgical biopsy.

Efficacy of pirfenidone for idiopathic pulmonary fibrosis: An Italian real life study.

Background: In this retrospective Italian study, which involved all major national interstitial lung diseases centers, we evaluated the effect of pirfenidone on disease progression in patients with IPF.

Methods: We retrospectively studied 128 patients diagnosed with mild, moderate or severe IPF, and the decline in lung function monitored during the one-year treatment with pirfenidone was compared with the decline measured during the one-year pre-treatment period.

Results: At baseline (first pirfenidone prescription), the mean percentage forced vital capacity (FVC) was 75% (35-143%) of predicted, and the mean percentage diffuse lung capacity (DLCO) was 47% (17-120%) of predicted.

HFE gene variants and iron-induced oxygen radical generation in idiopathic pulmonary fibrosis.

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).

An official American Thoracic Society statement: diagnosis and management of beryllium sensitivity and chronic beryllium disease.

Rationale: Beryllium continues to have a wide range of industrial applications. Exposure to beryllium can lead to sensitization (BeS) and chronic beryllium disease (CBD).

Objectives: The purpose of this statement is to increase awareness and knowledge about beryllium exposure, BeS, and CBD.

Idiopathic Pulmonary Fibrosis (IPF) incidence and prevalence in Italy.

Background: Studies of Idiopathic Pulmonary Fibrosis (IPF) epidemiology show regional variations of incidence and prevalence; no epidemiological studies have been carried out in Italy.

Objective: To determine incidence and prevalence rates of IPF in the population of a large Italian region.

Methods: in this cross-sectional study study data were collected on all patients of 18 years of age and older admitted as primary or secondary idiopathic fibrosing alveolitis (ICD9-CM 516.

Iron laden macrophages in idiopathic pulmonary fibrosis: the telltale of occult alveolar hemorrhage?

Rationale: A number of observations suggest that iron accumulates in the lungs of patients with idiopathic pulmonary fibrosis (IPF) with vascular abnormalities, including pulmonary hypertension.

Objectives: The aim of this study was to determine the prevalence and intensity of accumulation of alveolar epithelial lining fluid (ELF) iron and of alveolar macrophage hemosiderin in IPF and its relationship with disease severity.

Methods: Forty seven IPF patients and 14 healthy controls were retrospectively evaluated for iron accumulation in the lower respiratory tract using total iron spectrophotometric measures and for hemosiderin accumulation using the Perls' stain with the Golde score.

A 6MWT index to predict O2 flow correcting exercise induced SpO2 desaturation in ILD.

Introduction: Ambulatory oxygen (O2) is prescribed to interstitial lung disease (ILD) patients with mild hypoxemia, breathlessness and dyspnea on exertion. Oxygen titration is generally done with the 6 minute walk test (6MWT) to determine the O2 flow preventing oxygen saturation by pulse oximetry (SpO2) from falling below 88%. His study was designed to generate a 6MWT index predicting the O2 flow allowing completion of the 6MWT without oxygen desaturation.

Antigen-specific CD4- and CD8-positive signatures in different phases of Mycobacterium tuberculosis infection.

Current diagnostic standards for Mycobacterium tuberculosis (MTB) infection do not distinguish between active and latent tuberculosis (TB). To identify specific biomarkers characterizing the different forms of TB infection, we investigated in parallel with the QuantiFERON -TB Gold In-Tube (QFT-IT) the use of flow cytometry measuring CD4 and CD8 MTB-specific immune response in 17 active-TB patients, 21 health care workers (HCW), 14 recent contacts of TB patients (RC-TB), and 10 bacille Calmette Guerin (BCG)-vaccinated healthy controls (BCG-HC). A correlation (r = 0.

Epidemiology of idiopathic pulmonary fibrosis in Europe--an update.

Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterised by the formation of scar tissue within the lungs in the absence of any known cause. IPF is the most common of the idiopathic interstitial pneumonias and is an important cause of respiratory mortality. IPF is a relatively rare disease with an estimated prevalence ranging from two to 29 cases per 100,000 and slightly higher in men (20.

Awake thoracoscopic biopsy of interstitial lung disease.

Background: Surgical biopsy of interstitial lung disease (ILD) performed through general anesthesia and video-assisted thoracic surgery (VATS) is still associated with not negligible mortality and morbidity rates. We hypothesized feasibility and minimized side-effects of VATS lung biopsy performed by regional anesthesia methods in awake patients.

Methods: Thirty patients with clinical and radiologic diagnosis of undetermined ILD underwent awake VATS lung biopsy under thoracic epidural anesthesia (20 patients) or intercostal blocks (10 patients).

An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease.

Background: The clinical utility of bronchoalveolar lavage fluid (BAL) cell analysis for the diagnosis and management of patients with interstitial lung disease (ILD) has been a subject of debate and controversy. The American Thoracic Society (ATS) sponsored a committee of international experts to examine all relevant literature on BAL in ILD and provide recommendations concerning the use of BAL in the diagnosis and management of patients with suspected ILD.

Purpose: To provide recommendations for (1) the performance and processing of BAL and (2) the interpretation of BAL nucleated immune cell patterns and other BAL characteristics in patients with suspected ILD.

Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study.

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.

Randomized comparison of awake nonresectional versus nonawake resectional lung volume reduction surgery.

Objective: The study objective was to assess in a randomized controlled study (NCT00566839) the comparative results of awake nonresectional or nonawake resectional lung volume reduction surgery.

Method: Sixty-three patients were randomly assigned by computer to receive unilateral video-assisted thoracic surgery lung volume reduction surgery by a nonresectional technique performed through epidural anesthesia in 32 awake patients (awake group) or the standard resectional technique performed through general anesthesia in 31 patients (control group). Primary outcomes were hospital stay and changes in forced expiratory volume in 1 second.

Rescue of murine silica-induced lung injury and fibrosis by human embryonic stem cells.

Alveolar type II pneumocytes (ATII cells) are considered putative alveolar stem cells. Since no treatment is available to repair damaged epithelium and prevent lung fibrosis, novel approaches to induce regeneration of injured alveolar epithelium are desired. The objective of this study was to assess both the capacity of human embryonic stem cells (HUES-3) to differentiate in vitro into ATII cells and the ability of committed HUES-3 cells (HUES-3-ATII cells) to recover in vivo a pulmonary fibrosis model obtained by silica-induced damage.

Markers of anti-oxidant response in tobacco smoke exposed subjects: a data-mining review.

Tobacco smoke exposure is the cause of exaggerated inflammatory responses and tissue destruction leading to chronic bronchitis and emphysema. A number of studies have used biochemical and immunological technologies to identify biomarkers of severity, risk and pharmacological target of disease. Recently, genomic and proteomic studies have been carried out to explore tobacco smoke-induced lung damage mechanisms.

Serum CA 15-3 is increased in pulmonary fibrosis.

Background And Aim Of The Work: Carbohydrate antigen CA 15-3 is a glycoprotein whose expression, aberrant intracellular localization and changes in glycosylation have been associated with a wide range of cancers. Pulmonary fibrosis represents the final evolution of a chronic inflammation and is defined by the overgrowth of fibroblasts and exaggerated extracellular matrix deposition. The aim of the present study was to evaluate the possible diagnostic role of CA 15-3 in fibrosis in different idiopathic interstitial pneumonias.

Metabolic syndrome and risk of pulmonary involvement.

Metabolic syndrome (MS) is a complex disorder recognized clinically by the findings of abdominal obesity, elevated triglycerides, atherogenic dyslipidaemia, elevated blood pressure, high blood glucose and/or insulin resistance. It is associated with a pro-thrombotic and a pro-inflammatory state. A growing body of evidence suggests that individuals in the community with moderate airflow limitation may have co-existing systemic inflammation with this background.