Review Vascular remodelling in the pathogenesis of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible fibrosing interstitial pneumonia of unknown aetiology that usually leads to respiratory failure and death within 5 years of diagnosis. Alveolar epithelial cell injury, disruption of alveolar capillary membrane integrity and abnormal vascular repair and remodelling have all been proposed as possible pathogenic mechanisms. This review sum-marizes our current knowledge of the abnormalities in vascular remodelling observed in IPF and high-lights several of the cytokines thought to play a pathogenic role, which may ultimately prove to be future therapeutic targets