title: Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives.
abstract: Idiopathic pulmonary fibrosis (IPF) is a devastating condition with a 5-year survival of approximately 20%. The disease primarily occurs in elderly patients. IPF is a highly heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of rapid deterioration. In the last decade, improved understanding of disease mechanisms along with a more precise disease definition has allowed the design and completion of a number of high-quality clinical trials. Yet, until recently, IPF was essentially an untreatable disease. Finally, pirfenidone and nintedanib, two compounds with antifibrotic properties, have consistently proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough for patients and physicians alike, but there is still a long way to go. In fact, neither pirfenidone nor nintedanib is a cure for IPF, and most patients continue to progress despite treatment. As such, comprehensive care of patients with IPF, including management of comorbidities/complications and physical debility and timely referral for palliative care or, in a small number of highly selected patients, lung transplantation, remains essential. Several agents with high potential are currently being tested and many more are ready to be evaluated in clinical trials.
authors.affiliation: Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, 69126 Heidelberg, Germany; Translational Lung Research Center Heidelberg (TLRCH), Member of the German Center for Lung Research (DZL), 69126 Heidelberg, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Kreuter
authors.affiliation: Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, 45141 Essen, Germany.
authors.forename: Francesco
authors.initials: F
authors.lastname: Bonella
authors.affiliation: Department of Pulmonary Disease, Erasmus Medical Centre, University Hospital Rotterdam, 3015 CE Rotterdam, Netherlands.
authors.forename: Marlies
authors.initials: M
authors.lastname: Wijsenbeek
authors.affiliation: National Institute for Health Research Biological Research Unit, Royal Brompton Hospital, London SW3 6NP, UK; National Heart and Lung Institute, Imperial College, London SW3 6NP, UK.
authors.forename: Toby M
authors.initials: TM
authors.lastname: Maher
authors.affiliation: Medical University Clinic, Canton Hospital Baselland and University of Basel, 4410 Liestal, Switzerland.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
mesh_terms: D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D015897:Comorbidity; D019468:Disease Management; D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology; D007211:Indoles / Q000627:therapeutic use*; D011728:Pyridones / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib