The Management of Patients With Idiopathic Pulmonary Fibrosis

Frontiers in Medicine
Paolo SpagnoloFrancesco Bonella

Abstract

Idiopathic pulmonary fibrosis (IPF), the most common form of fibrosing idiopathic interstitial pneumonia, is an inexorably progressive disease with a 5-year survival of ~20%. In the last decade, our understanding of disease pathobiology has increased significantly and this has inevitably impacted on the approach to treatment. Indeed, the paradigm shift from a chronic inflammatory disorder to a primarily fibrotic one coupled with a more precise disease definition and redefined diagnostic criteria have resulted in a massive increase in the number of clinical trials evaluating novel candidate drugs. Most of these trials, however, have been negative, probably because of the multitude and redundancy of cell types, growth factors and profibrotic pathways involved in disease pathogenesis. As a consequence, until recently IPF has lacked effective therapies. Finally, in 2014, two large phase 3 clinical trials have provided robust evidence that pirfenidone, a compound with anti-fibrotic, anti-oxidant and anti-inflammatory properties, and nintedanib, a tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor, platelet-derived growth factor and fibroblast growth factor receptors are able to slow down functional dec...Continue Reading

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Methods Mentioned

BETA
biopsy
X-ray
bronchoalveolar lavage

Clinical Trials Mentioned

NCT01982968
NCT02611167
NCT02257177
NCT02550873
NCT02688647
NCT02503657
NCT02538536
NCT02738801
NCT03142191
NCT01766817

Software Mentioned

CAPACITY
TOMORROW

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