title: Idiopathic pulmonary fibrosis: the turning point is now!
abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with poor survival. Recent studies have improved understanding of IPF and new discoveries have led to novel treatment options, which now have become available for patients. In face of the newly available therapies we present an update on the pathophysiology and epidemiology of IPF. We discuss the typical clinical findings and elaborate diagnostic procedures according to current guidelines and our daily practice approach. The role of biomarkers will briefly be outlined. Finally, we discuss novel antifibrotic treatment options for IPF (pirfenidone, nintedanib) and the management of patients regarding to comorbidities and complications. Both pirfenidone and nintedanib were shown to reduce the progression of IPF and therefore represent novel therapeutic strategies in this so far untreatable chronic lung disease.
authors.affiliation: Department of Pulmonary Medicine, Inselspital, University Hospital Berne, Switzerland.
authors.forename: Manuela
authors.initials: M
authors.lastname: Funke
authors.affiliation: Department of Pulmonary Medicine, Inselspital, University Hospital Berne, Switzerland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Geiser
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D015415:Biomarkers; D018450:Disease Progression; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D005764:Gastroesophageal Reflux / Q000150:complications / Q000188:drug therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000628:therapy; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D007211:Indoles / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D011728:Pyridones / Q000627:therapeutic use
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D015415:Biomarkers; D004791:Enzyme Inhibitors; D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib