New tale for an old fox in IPF?

摘要

idiopathic pulmonary fibrosis (IPF) is a fatal progressive disease, with a poorly understood etiology and pathogene-sis.- Current estimates propose that IPF affects about 500,000 people in the USA and Europe with an increasing incidence. Histologically IPF is characterized by alveolar epithelial cell injury and activation, the formation of fibroblastic foci, and excessive matrix deposition in the lung parenchyma, leading to decreased lung function and ultimately respiratory failure. Most patients present with established disease with an average survival of less than 3 years from diagnosis (reviewed in Ref. 5). The insidious nature of the disease, combined with an incomplete understanding of pathogene-sis, accounts for the paucity of currently available therapeutic interventions.