Recent Advances and Future Needs in Interstitial Lung Diseases

Mark G. Jones; Luca Richeldi

doi:10.1055/s-0036-1580688

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2016; 37(03): 477-484
DOI: 10.1055/s-0036-1580688

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Recent Advances and Future Needs in Interstitial Lung Diseases

Mark G. Jones

¹National Institute for Health Research Southampton Respiratory Biomedical Research Unit and Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom

,

Luca Richeldi

¹National Institute for Health Research Southampton Respiratory Biomedical Research Unit and Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom

› Institutsangaben

Abstract

Interstitial lung diseases (ILDs) are a diverse range of conditions affecting the lung interstitium. The prototypic ILD, idiopathic pulmonary fibrosis (IPF), is a chronic progressive fibrotic lung disease with a median survival of only 3 years from the time of diagnosis. Recently significant progress has been made in both our understanding of the pathogenesis and of the therapeutic targeting of IPF. This culminated in the worldwide approval of the first antifibrotic therapies nintedanib and pirfenidone. While an important first step, patients continue to progress and better therapies are urgently required. The aim of this article is to highlight some of the recent advances that have been made in our understanding of genetics, disease classification, clinical trial design, and novel antifibrotic therapy in IPF. It discusses future priorities if we are to continue to increase the length and quality of life of patients with IPF, and considers possible approaches to translate the progress made in IPF to other progressive fibrotic lung diseases where our understanding remains limited.

Keywords

interstitial lung disease - idiopathic pulmonary fibrosis - genetics - antifibrotic therapy - pirfenidone - nintedanib

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Referenzen

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