Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players
Interstitial lung diseases (ILDs) that are known as diffuse parenchymal lung diseases (DPLDs) lead to the damage of alveolar epithelium and lung parenchyma, culminating in inflammation and widespread fibrosis. ILDs that account for more than 200 different pathologies can be divided into two groups:...
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Format: | Article |
Language: | English |
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MDPI AG
2021-08-01
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Series: | International Journal of Molecular Sciences |
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Online Access: | https://www.mdpi.com/1422-0067/22/16/8952 |
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doaj-6c75d730686c4566add0270c3d2e0504 |
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record_format |
Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Anna Valeria Samarelli Roberto Tonelli Alessandro Marchioni Giulia Bruzzi Filippo Gozzi Dario Andrisani Ivana Castaniere Linda Manicardi Antonio Moretti Luca Tabbì Stefania Cerri Bianca Beghè Massimo Dominici Enrico Clini |
spellingShingle |
Anna Valeria Samarelli Roberto Tonelli Alessandro Marchioni Giulia Bruzzi Filippo Gozzi Dario Andrisani Ivana Castaniere Linda Manicardi Antonio Moretti Luca Tabbì Stefania Cerri Bianca Beghè Massimo Dominici Enrico Clini Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players International Journal of Molecular Sciences lung disease idiopathic pulmonary fibrosis myofibroblast extracellular matrix proteins tgfβ1 signalling |
author_facet |
Anna Valeria Samarelli Roberto Tonelli Alessandro Marchioni Giulia Bruzzi Filippo Gozzi Dario Andrisani Ivana Castaniere Linda Manicardi Antonio Moretti Luca Tabbì Stefania Cerri Bianca Beghè Massimo Dominici Enrico Clini |
author_sort |
Anna Valeria Samarelli |
title |
Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players |
title_short |
Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players |
title_full |
Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players |
title_fullStr |
Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players |
title_full_unstemmed |
Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players |
title_sort |
fibrotic idiopathic interstitial lung disease: the molecular and cellular key players |
publisher |
MDPI AG |
series |
International Journal of Molecular Sciences |
issn |
1661-6596 1422-0067 |
publishDate |
2021-08-01 |
description |
Interstitial lung diseases (ILDs) that are known as diffuse parenchymal lung diseases (DPLDs) lead to the damage of alveolar epithelium and lung parenchyma, culminating in inflammation and widespread fibrosis. ILDs that account for more than 200 different pathologies can be divided into two groups: ILDs that have a known cause and those where the cause is unknown, classified as idiopathic interstitial pneumonia (IIP). IIPs include idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) known also as bronchiolitis obliterans organizing pneumonia (BOOP), acute interstitial pneumonia (AIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and lymphocytic interstitial pneumonia (LIP). In this review, our aim is to describe the pathogenic mechanisms that lead to the onset and progression of the different IIPs, starting from IPF as the most studied, in order to find both the common and standalone molecular and cellular key players among them. Finally, a deeper molecular and cellular characterization of different interstitial lung diseases without a known cause would contribute to giving a more accurate diagnosis to the patients, which would translate to a more effective treatment decision. |
topic |
lung disease idiopathic pulmonary fibrosis myofibroblast extracellular matrix proteins tgfβ1 signalling |
url |
https://www.mdpi.com/1422-0067/22/16/8952 |
work_keys_str_mv |
AT annavaleriasamarelli fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT robertotonelli fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT alessandromarchioni fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT giuliabruzzi fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT filippogozzi fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT darioandrisani fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT ivanacastaniere fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT lindamanicardi fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT antoniomoretti fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT lucatabbi fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT stefaniacerri fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT biancabeghe fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT massimodominici fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers AT enricoclini fibroticidiopathicinterstitiallungdiseasethemolecularandcellularkeyplayers |
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1721192484444307456 |
spelling |
doaj-6c75d730686c4566add0270c3d2e05042021-08-26T13:53:24ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-08-01228952895210.3390/ijms22168952Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key PlayersAnna Valeria Samarelli0Roberto Tonelli1Alessandro Marchioni2Giulia Bruzzi3Filippo Gozzi4Dario Andrisani5Ivana Castaniere6Linda Manicardi7Antonio Moretti8Luca Tabbì9Stefania Cerri10Bianca Beghè11Massimo Dominici12Enrico Clini13Laboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyRespiratory Diseases Unit, Department of Medical and Surgical Sciences, University Hospital of Modena, University of Modena Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyLaboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, ItalyInterstitial lung diseases (ILDs) that are known as diffuse parenchymal lung diseases (DPLDs) lead to the damage of alveolar epithelium and lung parenchyma, culminating in inflammation and widespread fibrosis. ILDs that account for more than 200 different pathologies can be divided into two groups: ILDs that have a known cause and those where the cause is unknown, classified as idiopathic interstitial pneumonia (IIP). IIPs include idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) known also as bronchiolitis obliterans organizing pneumonia (BOOP), acute interstitial pneumonia (AIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and lymphocytic interstitial pneumonia (LIP). In this review, our aim is to describe the pathogenic mechanisms that lead to the onset and progression of the different IIPs, starting from IPF as the most studied, in order to find both the common and standalone molecular and cellular key players among them. Finally, a deeper molecular and cellular characterization of different interstitial lung diseases without a known cause would contribute to giving a more accurate diagnosis to the patients, which would translate to a more effective treatment decision.https://www.mdpi.com/1422-0067/22/16/8952lung diseaseidiopathic pulmonary fibrosismyofibroblastextracellular matrix proteinstgfβ1 signalling |