Interstitial Lung Disorder

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Federico Lavorini - One of the best experts on this subject based on the ideXlab platform.

  • The pathogenetic mechanisms of cough in idiopathic pulmonary fibrosis
    Internal and Emergency Medicine, 2019
    Co-Authors: Elena Bargagli, Maria Masi, Marco Perruzza, Lucia Vietri, Laura Bergantini, Elena Torricelli, Giulia Biadene, Giovanni Fontana, Federico Lavorini
    Abstract:

    Idiopathic pulmonary fibrosis is a peripheral subpleural Interstitial Lung Disorder limited to the Lung not involving the airways. It has a poor prognosis (survival less than 5 years) and commonly an Interstitial pneumonia radiological pattern. Patients complain of a chronic dry cough in 80% of cases. A cough is often the first symptom of this rare disease, preceding dyspnea by years, and is associated with a poor prognosis, high dyspnea scores and low FVC percentages. The pathogenetic mechanisms leading to coughing in IPF are unclear. This review focuses on recent evidence of cough pathophysiology in this disease. Gastroesophageal reflux may promote coughing in IPF patients; bile salts and pepsin may be abundant in BAL of these patients, inducing overproduction of TGF-β by airway epithelial cells and mesenchymal transition with fibroblast recruitment/activation and extracellular matrix deposition. Patients have an enhanced cough reflex to capsaicin and substance P with respect to control subjects. Moreover, patients with the MUC5B polymorphism show more severe coughing as MUC5B encodes for the dominant mucin in the honeycomb cysts of IPF patients. Comorbidities, including asthma, gastroesophageal reflux, hypersensitivity pneumonitis, bronchiectasis, chronic obstructive pulmonary disease and emphysema, can induce coughing in IPF patients. There is no clear explanation of the causes of coughing in IPF. Further research into the pathophysiology of IPF and the pathogenetic mechanisms of coughing is necessary to improve survival and quality of life.

Jouke T Annema - One of the best experts on this subject based on the ideXlab platform.

  • confocal laser endomicroscopy as a guidance tool for transbronchial Lung cryobiopsies in Interstitial Lung Disorder
    Respiration, 2019
    Co-Authors: Lizzy Wijmans, Peter I Bonta, Rita Rochapinto, Daniel M De Bruin, Paul Brinkman, Rene E Jonkers, Joris J T H Roelofs, Venerino Poletti, Juergen Hetzel, Jouke T Annema
    Abstract:

    Background: Transbronchial cryobiopsy (TBCB) of the Lung parenchyma is a minimally invasive alternative for surgical Lung biopsy in Interstitial Lung disease (ILD) patients. Drawbacks are the nondiagnostic rate and complication risk of pneumothorax and bleeding. Fluoroscopy is the current guidance tool for TBCB, which is limited by 2D imaging and a radiation dose for the patient. Confocal laser endomicroscopy (CLE) is a high-resolution imaging technique that provides immediate feedback during bronchoscopy about the elastin fiber network of peripheral Lung areas. Both the visceral pleura and fibrotic Lung areas consist of elastin fibers and are therefore potentially detectable with CLE. Objectives: To investigate whether CLE is capable of (1) distinguishing fibrotic from normal alveolar areas and (2) identifying the pleura. Methods: In and ex vivo CLE imaging obtained during bronchoscopy was compared with histology of Lung biopsies in 14 ILD patients. Results: CLE imaging of the alveolar compartment was feasible in all patients without adverse events. Based on CLE imaging, key characteristics that influence both diagnostic yield (dense fibrotic areas) and complication rate (pleura and subpleural space) were visualized. Conclusions: CLE seems a promising alternative to fluoroscopy as a guidance tool for TBCB procedures.

Elena Bargagli - One of the best experts on this subject based on the ideXlab platform.

  • The pathogenetic mechanisms of cough in idiopathic pulmonary fibrosis
    Internal and Emergency Medicine, 2019
    Co-Authors: Elena Bargagli, Maria Masi, Marco Perruzza, Lucia Vietri, Laura Bergantini, Elena Torricelli, Giulia Biadene, Giovanni Fontana, Federico Lavorini
    Abstract:

    Idiopathic pulmonary fibrosis is a peripheral subpleural Interstitial Lung Disorder limited to the Lung not involving the airways. It has a poor prognosis (survival less than 5 years) and commonly an Interstitial pneumonia radiological pattern. Patients complain of a chronic dry cough in 80% of cases. A cough is often the first symptom of this rare disease, preceding dyspnea by years, and is associated with a poor prognosis, high dyspnea scores and low FVC percentages. The pathogenetic mechanisms leading to coughing in IPF are unclear. This review focuses on recent evidence of cough pathophysiology in this disease. Gastroesophageal reflux may promote coughing in IPF patients; bile salts and pepsin may be abundant in BAL of these patients, inducing overproduction of TGF-β by airway epithelial cells and mesenchymal transition with fibroblast recruitment/activation and extracellular matrix deposition. Patients have an enhanced cough reflex to capsaicin and substance P with respect to control subjects. Moreover, patients with the MUC5B polymorphism show more severe coughing as MUC5B encodes for the dominant mucin in the honeycomb cysts of IPF patients. Comorbidities, including asthma, gastroesophageal reflux, hypersensitivity pneumonitis, bronchiectasis, chronic obstructive pulmonary disease and emphysema, can induce coughing in IPF patients. There is no clear explanation of the causes of coughing in IPF. Further research into the pathophysiology of IPF and the pathogenetic mechanisms of coughing is necessary to improve survival and quality of life.

  • Macrophage migration inhibitory factor in Lung tissue of idiopathic pulmonary fibrosis patients
    Experimental Lung Research, 2016
    Co-Authors: Carmela Olivieri, Elena Bargagli, Simona Inghilleri, Ilaria Campo, Marcella Cintorino, Paola Rottoli
    Abstract:

    ABSTRACTIntroduction: Idiopathic pulmonary fibrosis (IPF) is a severe Interstitial Lung Disorder characterized by a pattern of Usual Interstitial Pneumonia where the presence of fibroblastic foci is the hallmark of the disease. Aim of the Study: In the present study, we analyzed the migration inhibitory factor (MIF) expression in Lung tissue of IPF patients compared with healthy controls and organizing pneumonia (OP) patients focusing into MIF potential role in fibroblastic foci development. Materials and Methods: The immunohistochemical analysis was performed in 10 IPF patients (7 male), 3 OP patients (2 male), and 3 healthy controls (all male) using the streptavidin-biotin method (Dako). Results: In IPF samples, MIF resulted overexpressed in the areas of active fibrosis and, in particular, in the alveolar epithelium, bronchiolar epithelium, and in the peripheral zones of fibroblastic foci. Bronchiolar epithelium from organizing pneumonia patients resulted only weakly positive for MIF while no evidence o...

Lizzy Wijmans - One of the best experts on this subject based on the ideXlab platform.

  • confocal laser endomicroscopy as a guidance tool for transbronchial Lung cryobiopsies in Interstitial Lung Disorder
    Respiration, 2019
    Co-Authors: Lizzy Wijmans, Peter I Bonta, Rita Rochapinto, Daniel M De Bruin, Paul Brinkman, Rene E Jonkers, Joris J T H Roelofs, Venerino Poletti, Juergen Hetzel, Jouke T Annema
    Abstract:

    Background: Transbronchial cryobiopsy (TBCB) of the Lung parenchyma is a minimally invasive alternative for surgical Lung biopsy in Interstitial Lung disease (ILD) patients. Drawbacks are the nondiagnostic rate and complication risk of pneumothorax and bleeding. Fluoroscopy is the current guidance tool for TBCB, which is limited by 2D imaging and a radiation dose for the patient. Confocal laser endomicroscopy (CLE) is a high-resolution imaging technique that provides immediate feedback during bronchoscopy about the elastin fiber network of peripheral Lung areas. Both the visceral pleura and fibrotic Lung areas consist of elastin fibers and are therefore potentially detectable with CLE. Objectives: To investigate whether CLE is capable of (1) distinguishing fibrotic from normal alveolar areas and (2) identifying the pleura. Methods: In and ex vivo CLE imaging obtained during bronchoscopy was compared with histology of Lung biopsies in 14 ILD patients. Results: CLE imaging of the alveolar compartment was feasible in all patients without adverse events. Based on CLE imaging, key characteristics that influence both diagnostic yield (dense fibrotic areas) and complication rate (pleura and subpleural space) were visualized. Conclusions: CLE seems a promising alternative to fluoroscopy as a guidance tool for TBCB procedures.

S K Gupta - One of the best experts on this subject based on the ideXlab platform.