The changing treatment landscape in idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible fibrotic disease of the lung that has greatly frustrated clinicians for a long time. The prognosis of IPF (median survival 2-5 years following diagnosis) is poorer than that of some cancers and for many years no significant advances were made in its management. However, between 2011 and 2014 a number of pivotal developments were made that have improved the outlook for patients with IPF. Herein, we review this rapidly changing landscape, discussing key events whilst still acknowledging that IPF remains a challenging disease to diagnose and manage.
Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University Duisburg-Essen, Essen, Germany ulrich.costabel@ruhrlandklinik.uk-essen.de.
Ulrich
U
Costabel
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D005060:Europe; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy / Q000401:mortality; D017410:Practice Guidelines as Topic; D011728:Pyridones / Q000627:therapeutic use; D014481:United States
D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone