Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease, characterised by progressive scarring of the lung and associated with a high burden of disease and early death. The pathophysiological understanding, clinical diagnostics and therapy of IPF have significantly evolved in recent years. While the recent introduction of the two antifibrotic drugs pirfenidone and nintedanib led to a significant reduction in lung function decline, there is still no cure for IPF; thus, new therapeutic approaches are needed. Currently, several clinical phase I–III trials are focusing on novel therapeutic targets. Furthermore, new approaches in nonpharmacological treatments in palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities and acute exacerbations aim to improve symptom control and quality of life. Here we summarise new therapeutic attempts and potential future approaches to treat this devastating disease.
Abstract
There is still no cure for IPF. Clinical trials focus on new therapeutic targets, improvements in nonpharmacological therapies and treatment of comorbidities and acute exacerbations. All future therapies should aim to reduce the burden of disease. http://bit.ly/2XdxqAP
Footnotes
Publication of this peer-reviewed article was sponsored by Boehringer Ingelheim, Germany (principal sponsor European Respiratory Review issue 153).
Conflict of interest: V. Somogyi has nothing to disclose.
Conflict of interest: N. Chaudhuri has nothing to disclose.
Conflict of interest: S.E. Torrisi has nothing to disclose.
Conflict of interest: N. Kahn has nothing to disclose.
Conflict of interest: V. Müller has nothing to disclose.
Conflict of interest: M. Kreuter has nothing to disclose.
- Received February 27, 2019.
- Accepted May 16, 2019.
- Copyright ©ERS 2019.
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.