The therapy of idiopathic pulmonary fibrosis: what is next?

Autor:	Somogyi V; Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany.; Dept of Pulmonology, Semmelweis University, Budapest, Hungary., Chaudhuri N; Manchester University NHS Foundation Trust, Wythenshawe Hospital, Manchester, UK., Torrisi SE; Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany.; Regional Referral Centre for Rare Lung Diseases, University Hospital 'Policlinico', Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy., Kahn N; Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany., Müller V; Dept of Pulmonology, Semmelweis University, Budapest, Hungary., Kreuter M; Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany kreuter@uni-heidelberg.de.
Jazyk:	angličtina
Zdroj:	European respiratory review : an official journal of the European Respiratory Society [Eur Respir Rev] 2019 Sep 04; Vol. 28 (153). Date of Electronic Publication: 2019 Sep 04 (Print Publication: 2019).
DOI:	10.1183/16000617.0021-2019
Abstrakt:	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. Competing Interests: 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. (Copyright ©ERS 2019.)
Databáze:	MEDLINE
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