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Minerva Pneumologica 2016 December;55(4):119-31

Copyright © 2016 EDIZIONI MINERVA MEDICA

language: English

Overview on diagnosis and treatment of idiopathic pulmonary fibrosis

Davide BIONDINI, Elisabetta BALESTRO, Paolo SPAGNOLO

Section of Respiratory Diseases, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy


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Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. Making an accurate diagnosis is challenging, but it is crucial since, following almost two decades of disappointing results, two compounds with pleiotropic mechanisms of action, pirfenidone and nintedanib, have proven to be effective in slowing down functional decline and disease progression in patients with IPF. Despite this major advance for patients and physicians alike, it is worth remembering that available drugs neither stop disease progression nor improve lung function. Following a massive effort of scientists, patients and industry, a number of new agents with high potential have been identified and are either currently being tested or ready for clinical trials. This article seeks to provide an update on diagnosis and treatment of IPF, focusing on newly available antifibrotic drugs.

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