FIBROSIS · Fibroproliferative Invasive Fibroblasts in Idiopathic Pulmonary Fibrosis

Fibroproliferative Invasive Fibroblasts in Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that kills more people each year than most cancers. Scarring occurs in IPF patients' lungs as the disease progresses make it increasingly difficult to breath and carry out normal tasks. There are limited therapies for patients with IPF and none that can cure the disease fully. This makes IPF an important disease to research. In a healthy lung, epithelial cells line the surfaces in contact with the air and a cell type called a fibroblast make the glue and creates the scaffolding, composed of extracellular matrix (ECM) proteins, for other cells. In IPF patients it is believed that the airway lining cells become injured. For an unknown reason the normal repair response, led by fibroblasts, becomes dysfunctional. Instead of a neat repair to the damaged area the fibroblasts become highly active, increase in number, and produce large amounts of ECM glue and scaffolding leading to the spread of thick and stiff scar tissue. This scarring makes it difficult for the lung to function normally and can ultimately be fatal. Lung scarring can occur in other lung diseases like Systemic Sclerosis, and most recently in some patients recovering from COVID-19 infection. To understand why the normal repair process goes wrong and results in progressive scarring of the lung in diseases like IPF we must first understand the cells involved. The latest research suggests that many types of the key repair cell type, fibroblasts, exist in our lungs. Studying this fibroblasts and how they contribute to scarring will benefits not just patients with IPF but also patients with others conditions in which scarring occurs. This project tackled the question of whether specific fibroblast sub-types might be more involved in the abnormal repair process than others and examined the role of key, known, fibroblast sub-types. The objectives of this project were: (1) Study the characteristics, genetics and behavior of key fibroblast sub-types to identify ways in which they contribute to disease. (2) Use the very latest technologies to definitively identify the different fibroblast sub-types in the lung. (3) Study disease models of lung scarring to try and understand what fibroblast sub-type, if any, is the major driver of disease and lung tissue damage.

Data: CORDIS, © European Union