title: An update on idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic disease with a median survival of 3-5 years. Despite recent advances the pathophysiology of the disease remains not fully understood. However, injury of type II alveolar epithelial cells is considered the key event for the initiation of the development of fibrosis. An accurate diagnosis is imperative because commencing treatment at an early stage may reduce disease progression. In this regard, the multidisciplinary disease meeting between pulmonologists, radiologists and pathologists has definitely improved the diagnostic confidence. Importantly, a milestone has been recently reached as the first IPF-specific drug namely pirfenidone has been licensed in Europe, Japan and Asia.
authors.affiliation: Interstitial Lung Disease Unit, University Hospital of Heraklion, Crete, Greece. gmargaritop@yahoo.gr
authors.forename: G A
authors.initials: GA
authors.lastname: Margaritopoulos
authors.forename: I
authors.initials: I
authors.lastname: Giannarakis
authors.forename: N M
authors.initials: NM
authors.lastname: Siafakas
authors.forename: K M
authors.initials: KM
authors.lastname: Antoniou
mesh_terms: D000818:Animals; D042241:Early Diagnosis; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D008168:Lung / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D011237:Predictive Value of Tests; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use; D012307:Risk Factors
chemical_list: D011728:Pyridones; C093844:pirfenidone