Idiopathic pulmonary fibrosis [Fibrosi polmonare idiopatica]

Idiopathic pulmonary fibrosis (EPF) is a fibrosing interstitial pneumonia of unknown etiology characterised by poor prognosis and a morphologic pattern of usual interstitial pneumonia (UIP). Although its pathogenesis is unclear, today it is considered to be the result of an anomalous wound healing to repeated alveolar epithelial insult, rather than a purely inflammatory disease. New treatments that aim to prevent or block flbroproliferative processes and/or increase alveolar re-epithelisation are, therefore, the subject of clinical controlled trials. Lung transplant is currently the only therapy demonstrated to modify the prognosis of IPF. However, it seems likely that some of the molecules being tested will prove to counteract the course of this complex disease. Early diagnosis is, therefore, important and patients should be referred to specialised centres where the best therapy can be decided.