Dysregulated balance of lung macrophage populations in idiopathic pulmonary fibrosis revealed by single-cell RNA seq: an unstable “ménage-à-trois”

摘要

Unravelling the cellular and molecular mechanisms associated with the initiation and progression of idiopathic pulmonary fibrosis (IPF) represents a huge challenge. A better knowledge of these issues is not only imperative for elucidating the pathogenic mechanisms of IPF but also to provide new therapeutic strategies as well as new options for diagnosis and prognosis. The pathogenesis of IPF is particularly complex and involves multiple cell types associated within a dynamic pathobiological process, involving excessive wound healing with chronic inflammation, apoptosis of alveolar epithelial and endothelial cells, formation of honeycomb cysts composed of pseudostratified and bronchiolar-like epithelium, activation of myofibroblast effector cells with the formation of fibroblasts foci, and, finally, excessive deposition of extracellular matrix resulting in the destruction of the lung architecture and the loss of lung functions. Genetic susceptibility, ageing, and exposure to inhaled toxins all contribute to the initiation of the fibrotic process [1–3].