3D In Vitro Models: Novel Insights into Idiopathic Pulmonary Fibrosis Pathophysiology and Drug Screening
Ana Ivonne Vazquez-Armendariz,
Margarida Maria Barroso,
Elie El Agha,
Susanne Herold
Affiliations
Ana Ivonne Vazquez-Armendariz
Department of Medicine V, Internal Medicine, Infectious Diseases and Infection Control, Universities of Giessen and Marburg Lung Center (UGMLC), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Member of the German Center for Lung Research (DZL), Justus-Liebig University Giessen, 35392 Giessen, Hessen, Germany
Margarida Maria Barroso
Department of Medicine V, Internal Medicine, Infectious Diseases and Infection Control, Universities of Giessen and Marburg Lung Center (UGMLC), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Member of the German Center for Lung Research (DZL), Justus-Liebig University Giessen, 35392 Giessen, Hessen, Germany
Elie El Agha
Department of Medicine V, Internal Medicine, Infectious Diseases and Infection Control, Universities of Giessen and Marburg Lung Center (UGMLC), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Member of the German Center for Lung Research (DZL), Justus-Liebig University Giessen, 35392 Giessen, Hessen, Germany
Susanne Herold
Department of Medicine V, Internal Medicine, Infectious Diseases and Infection Control, Universities of Giessen and Marburg Lung Center (UGMLC), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Member of the German Center for Lung Research (DZL), Justus-Liebig University Giessen, 35392 Giessen, Hessen, Germany
Idiopathic pulmonary fibrosis (IPF) is a progressive and often lethal interstitial lung disease of unknown aetiology. IPF is characterised by myofibroblast activation, tissue stiffening, and alveolar epithelium injury. As current IPF treatments fail to halt disease progression or induce regeneration, there is a pressing need for the development of novel therapeutic targets. In this regard, tri-dimensional (3D) models have rapidly emerged as powerful platforms for disease modelling, drug screening and discovery. In this review, we will touch on how 3D in vitro models such as hydrogels, precision-cut lung slices, and, more recently, lung organoids and lung-on-chip devices have been generated and/or modified to reveal distinct cellular and molecular signalling pathways activated during fibrotic processes. Markedly, we will address how these platforms could provide a better understanding of fibrosis pathophysiology and uncover effective treatment strategies for IPF patients.