An update on idiopathic pulmonary fibrosis
Margaritopoulos, G.A.; Giannarakis, I.; Siafakas, N.M.; Antoniou, K.M.
Panminerva Medica 55(2): 109-120
2013
ISSN/ISBN: 0031-0808 PMID: 23676953 Document Number: 669745
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.