Epithelium-dependent profibrotic milieu in the pathogenesis of idiopathic pulmonary fibrosis: current status and future directions

摘要

Background and AimIdiopathic pulmonary fibrosis (IPF) is characterized by hyperplasia of type II alveolar epithelial cells, aggregation of activated (myo)fibroblasts and excessive deposition of extracellular matrix, which will ultimately lead to lung architecture destruction with no proven effective therapies. Despite a significant increase in our understanding on the etiology and pathogenesis of IPF, the real triggers that initiate epithelial cell injury and promote fibrosis evolution are still elusive. We wanted to discuss the evolution of hypothesis on IPF pathogenesis and to suggest some new directions which need to be further elucidated.