2. Academic Validation
  3. Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players

Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players

  • Int J Mol Sci. 2021 Aug 19;22(16):8952. doi: 10.3390/ijms22168952.
Anna Valeria Samarelli 1 2,  Roberto Tonelli 1 2 3,  Alessandro Marchioni 1 2,  Giulia Bruzzi 1 2,  Filippo Gozzi 1 2 3,  Dario Andrisani 1 2 3,  Ivana Castaniere 1 2 3,  Linda Manicardi 1 2,  Antonio Moretti 1 2,  Luca Tabbì 2,  Stefania Cerri 1 2,  Bianca Beghè 1 2,  Massimo Dominici 1 4,  Enrico Clini 1 2
Affiliations

Affiliations

  • 1 Laboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, 41100 Modena, Italy.
  • 2 Respiratory Diseases Unit, Department of Medical and Surgical Sciences, University Hospital of Modena, University of Modena Reggio Emilia, 41100 Modena, Italy.
  • 3 Clinical and Experimental Medicine PhD Program, University of Modena Reggio Emilia, 41100 Modena, Italy.
  • 4 Oncology Unit, University Hospital of Modena, University of Modena and Reggio Emilia, 41100 Modena, Italy.
PMID: 34445658 DOI: 10.3390/ijms22168952
Abstract

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.

Keywords

extracellular matrix proteins; idiopathic pulmonary fibrosis; lung disease; myofibroblast; tgfβ1 signalling.

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