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. 2023 Apr 25;24(9):7849.
doi: 10.3390/ijms24097849.

Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review

Francesco Amati  1   2 , Anna Stainer  1   2 , Veronica Polelli  2 , Marco Mantero  3   4 , Andrea Gramegna  3   4 , Francesco Blasi  3   4 , Stefano Aliberti  1   2
Affiliations

Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review

Francesco Amati et al. Int J Mol Sci. .

Abstract

Pirfenidone and nintedanib are antifibrotic medications approved for idiopathic pulmonary fibrosis treatment by regulatory agencies and available for clinical use worldwide. These drugs have been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with idiopathic pulmonary fibrosis. Recent data suggest that different interstitial lung diseases with a progressive pulmonary fibrosis phenotype can share similar pathogenetic and biological pathways and could be amenable to antifibrotic therapies. Indeed, historical management strategies in interstitial lung disease have failed to identify potential treatments once progression has occurred despite available drugs. In this systematic review, we summarized data on the efficacy of pirfenidone and nintedanib in interstitial lung diseases other than idiopathic pulmonary fibrosis as well as ongoing and upcoming clinical trials. We identify two well-designed trials regarding nintedanib demonstrating the efficacy of this drug in slowing disease progression in patients with interstitial lung diseases other than idiopathic pulmonary fibrosis. On the other hand, results on the use of pirfenidone in interstitial lung diseases other than idiopathic pulmonary fibrosis should be interpreted with more caution on the basis of trial limitations. Several randomized control trials are underway to improve the quality of evidence in the interstitial lung disease field.

Keywords: efficacy; forced vital capacity; interstitial lung diseases; nintedanib; pirfenidone.

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Conflict of interest statement

Amati reports personal fees from Boehringer and personal fees from Insmed outside the submitted work. Stainer has nothing to disclose. Gramegna reports personal fees from Astrazeneca, personal fees from Chiesi, personal fees from Grifols, personal fees from Insmed, personal fees from Menarini, personal fees from Vertex, personal fees from Zambon, outside the submitted work. Aliberti reports personal fees from Bayer Healthcare, personal fees from Grifols, personal fees from Astra Zeneca, personal fees from Zambon, grants and personal fees from Chiesi, grants and personal fees from INSMED, personal fees from GlaxoSmithKline, personal fees from Menarini, personal fees from ZetaCube Srl, and grants from Fisher & Paykel outside the submitted work. Mantero has nothing to disclose. Dr. Polelli has nothing to disclose. Blasi reports grants and personal fees from Astrazeneca, personal fees from Chiesi, personal fees from GlaxoSmithKline, personal fees from Grifols, personal fees from Guidotti, grants and personal fees from Insmed, personal fees from Novartis, personal fees from Om Pharma, personal fees from Pfizer, personal fees from Sanofi, personal fees from Vertex, personal fees from Viatris, and personal fees from Zambon outside the submitted work.

Figures

Figure 1
Figure 1
Flow chart of the systematic review.
See this image and copyright information in PMC

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