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BioMed Research International
Volume 2015, Article ID 329481, 10 pages
http://dx.doi.org/10.1155/2015/329481
Review Article

Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives

1Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, 69126 Heidelberg, Germany
2Translational Lung Research Center Heidelberg (TLRCH), Member of the German Center for Lung Research (DZL), 69126 Heidelberg, Germany
3Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, 45141 Essen, Germany
4Department of Pulmonary Disease, Erasmus Medical Centre, University Hospital Rotterdam, 3015 CE Rotterdam, Netherlands
5National Institute for Health Research Biological Research Unit, Royal Brompton Hospital, London SW3 6NP, UK
6National Heart and Lung Institute, Imperial College, London SW3 6NP, UK
7Medical University Clinic, Canton Hospital Baselland and University of Basel, 4410 Liestal, Switzerland

Received 4 September 2015; Accepted 11 November 2015

Academic Editor: Natale Daniele Brunetti

Copyright © 2015 Michael Kreuter et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.