Abstract
Interstitial lung diseases (ILDs) are a large and diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most common and best-studied member. Increasing interest in fibrosis as a therapeutic target and the appreciation that fibrotic mechanisms may be a treatable target of IPF prompted the development and subsequent approval of the antifibrotics, pirfenidone and nintedanib. The management of ILDs has changed considerably following an understanding that IPF and some ILDs share similar disease behavior of progressive fibrosis, termed “progressive fibrosing phenotype”. Indeed, antifibrotic treatment has shown to be beneficial in ILDs characterized by the progressive fibrosing phenotype. This narrative review summarizes current knowledge in the field of progressive fibrosing ILDs. Here, we discuss the clinical characteristics and pathogenesis of lung fibrosis and highlight relevant literature concerning the mechanisms underlying progressive fibrosing ILDs. We also summarize current diagnostic approaches and the available treatments of progressive fibrosing ILDs and address the optimization of treating progressive fibrosing ILDs with antifibrotics in clinical practice.
Highlights
Pirfenidone, an inhibitor of transforming growth factor-ß (TGFß), and nintedanib, a tyrosine kinase inhibitor, were the first drugs to be approved by the European Medicines Agency (EMA) and the Food and Drug Administration (FDA) for the treatment of idiopathic pulmonary fibrosis (IPF), a chronic and fatal interstitial lung disease (ILD)
The results of these trials showed that the antifibrotic, nintedanib, significantly reduced the decline of lung function compared with placebo and to a similar extent to that observed in patients with IPF [12,13], resulting in new licensed indications of antifibrotics
This study reported that machine learning-based quantification of fine crackles, which are frequently heard in patients with ILDs, can predict the high resolution computed tomography (HRCT) findings of lung fibrosis, thereby supporting the timely and sensitive diagnosis of ILDs
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. The phase 2b study with pirfenidone in unclassifiable ILD (a subset of ILDs that are unable to be classified according to the current diagnostic framework, see O’Callaghan et al for a recent comprehensive review [14]) did not reach its primary endpoint; its results are only suggestive and not conclusive [15] The results of these trials showed that the antifibrotic, nintedanib, significantly reduced the decline of lung function compared with placebo and to a similar extent to that observed in patients with IPF [12,13], resulting in new licensed indications of antifibrotics.
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