The therapy of idiopathic pulmonary fibrosis: what is next?

V Somogyi, N Chaudhuri, SE Torrisi… - European …, 2019 - Eur Respiratory Soc
V Somogyi, N Chaudhuri, SE Torrisi, N Kahn, V Müller, M Kreuter
European Respiratory Review, 2019Eur Respiratory Soc
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 …
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.
European Respiratory Society