Role of Wnt/PCP pathway on bronchial cell function in Idiopathic Pulmonary Fibrosis

Pneumologie ◽  
2011 ◽  
Vol 65 (12) ◽  
Author(s):  
S Barkha ◽  
M Gegg ◽  
H Lickert ◽  
M Königshoff
Pneumologie ◽  
2014 ◽  
Vol 68 (06) ◽  
Author(s):  
S Skwarna ◽  
I Henneke ◽  
W Seeger ◽  
T Geiser ◽  
A Günther ◽  
...  

2019 ◽  
Vol 13 (3) ◽  
pp. 166-173 ◽  
Author(s):  
Sergio Harari ◽  
Antonella Caminati ◽  
Marco Confalonieri ◽  
Venerino Poletti ◽  
Carlo Vancheri ◽  
...  

2013 ◽  
Vol 6 (1) ◽  
pp. 20 ◽  
Author(s):  
Deborah L Clarke ◽  
Alan M Carruthers ◽  
Tomas Mustelin ◽  
Lynne A Murray

2011 ◽  
Vol 135 (6) ◽  
pp. 780-788 ◽  
Author(s):  
Robert J. Homer ◽  
Jack A. Elias ◽  
Chun Gun Lee ◽  
Erica Herzog

Abstract Context.—Idiopathic pulmonary fibrosis is a uniformly lethal disease with limited biomarkers and no proven therapeutic intervention short of lung transplantation. Pulmonary fibrosis at one time was thought to be a result of inflammation in the lung. Although some forms of pulmonary fibrosis may result from inflammation, idiopathic pulmonary fibrosis is currently thought to result from cell death primarily and inflammation secondarily. Objective.—To determine the role of inflammation in pulmonary fibrosis in light of our laboratory's published and unpublished research and published literature. Data Sources.—Review based on our laboratory's published and unpublished experimental data with relevant background and clinical context provided. Conclusions.—Although cell death is central to pulmonary fibrosis, the proper cytokine environment leading to macrophage polarization is also critical. Evaluation of this environment is promising both for the development of disease biomarkers and for targets for therapeutic intervention.


2021 ◽  
pp. 2101372
Author(s):  
Taro Yasuma ◽  
Corina N. D'Alessandro-Gabazza ◽  
Osamu Hataji ◽  
Tetsu Kobayashi ◽  
Esteban C Gabazza

2021 ◽  
Vol 12 ◽  
Author(s):  
Chiel van Geffen ◽  
Astrid Deißler ◽  
Markus Quante ◽  
Harald Renz ◽  
Dominik Hartl ◽  
...  

The immune system is receiving increasing attention for interstitial lung diseases, as knowledge on its role in fibrosis development and response to therapies is expanding. Uncontrolled immune responses and unbalanced injury-inflammation-repair processes drive the initiation and progression of idiopathic pulmonary fibrosis. The regulatory immune system plays important roles in controlling pathogenic immune responses, regulating inflammation and modulating the transition of inflammation to fibrosis. This review aims to summarize and critically discuss the current knowledge on the potential role of regulatory immune cells, including mesenchymal stromal/stem cells, regulatory T cells, regulatory B cells, macrophages, dendritic cells and myeloid-derived suppressor cells in idiopathic pulmonary fibrosis. Furthermore, we review the emerging role of regulatory immune cells in anti-fibrotic therapy and lung transplantation. A comprehensive understanding of immune regulation could pave the way towards new therapeutic or preventive approaches in idiopathic pulmonary fibrosis.


2021 ◽  
Author(s):  
Giovanna Elisiana Carpagnano ◽  
Piera Soccio ◽  
Giulia Scioscia ◽  
Grazia Pia Palladino ◽  
Maria Pia Foschino Barbaro ◽  
...  

2010 ◽  
Vol 134 (3) ◽  
pp. 462-480 ◽  
Author(s):  
Victor L. Roggli ◽  
Allen R. Gibbs ◽  
Richard Attanoos ◽  
Andrew Churg ◽  
Helmut Popper ◽  
...  

Abstract Asbestosis is defined as diffuse pulmonary fibrosis caused by the inhalation of excessive amounts of asbestos fibers. Pathologically, both pulmonary fibrosis of a particular pattern and evidence of excess asbestos in the lungs must be present. Clinically, the disease usually progresses slowly, with a typical latent period of more than 20 years from first exposure to onset of symptoms. Differential Diagnosis: Idiopathic Pulmonary Fibrosis The pulmonary fibrosis of asbestosis is interstitial and has a basal subpleural distribution, similar to that seen in idiopathic pulmonary fibrosis, which is the principal differential diagnosis. However, there are differences between the 2 diseases apart from the presence or absence of asbestos. First, the interstitial fibrosis of asbestosis is accompanied by very little inflammation, which, although not marked, is better developed in idiopathic pulmonary fibrosis. Second, in keeping with the slow tempo of the disease, the fibroblastic foci that characterize idiopathic pulmonary fibrosis are infrequent in asbestosis. Third, asbestosis is almost always accompanied by mild fibrosis of the visceral pleura, a feature that is rare in idiopathic pulmonary fibrosis. Differential Diagnosis: Respiratory Bronchiolitis Asbestosis is believed to start in the region of the respiratory bronchiole and gradually extends outward to involve more and more of the lung acinus, until the separate foci of fibrosis link, resulting in the characteristically diffuse pattern of the disease. These early stages of the disease are diagnostically problematic because similar centriacinar fibrosis is often seen in cigarette smokers and is characteristic of mixed-dust pneumoconiosis. Fibrosis limited to the walls of the bronchioles does not represent asbestosis. Role of Asbestos Bodies Histologic evidence of asbestos inhalation is provided by the identification of asbestos bodies either lying freely in the air spaces or embedded in the interstitial fibrosis. Asbestos bodies are distinguished from other ferruginous bodies by their thin, transparent core. Two or more asbestos bodies per square centimeter of a 5-μm-thick lung section, in combination with interstitial fibrosis of the appropriate pattern, are indicative of asbestosis. Fewer asbestos bodies do not necessarily exclude a diagnosis of asbestosis, but evidence of excess asbestos would then require quantitative studies performed on lung digests. Role of Fiber Analysis Quantification of asbestos load may be performed on lung digests or bronchoalveolar lavage material, employing either light microscopy, scanning electron microscopy, or transmission electron microscopy. Whichever technique is employed, the results are only dependable if the laboratory is well practiced in the method chosen, frequently performs such analyses, and the results are compared with those obtained by the same laboratory applying the same technique to a control population.


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