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Review
. 2025 Jun;11(2):195-234.
doi: 10.1007/s41030-025-00296-0. Epub 2025 May 5.

Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons

Giacomo Giulianelli  1 , Elisabetta Cocconcelli  2 , Giordano Fiorentù  2 , Nicol Bernardinello  2 , Elisabetta Balestro  2 , Paolo Spagnolo  2
Affiliations
Review

Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons

Giacomo Giulianelli et al. Pulm Ther. 2025 Jun.

Abstract

Idiopathic pulmonary fibrosis (IPF) represents a clinical and therapeutic challenge characterized by progressive fibrosis and destruction of the lung architecture. The pathogenesis of IPF has been long debated; while it is generally believed that repeated lung injury and abnormal wound repair are the main pathogenetic mechanisms, clear understanding of disease development and efficacious treatment remain important unmet needs. Indeed, current standard of care (i.e., the antifibrotic drugs pirfenidone and nintedanib) can slow down lung function decline and disease progression without halting the disease. In the last 2 decades, several clinical trials in IPF have been completed mostly with negative results. Yet, unprecedented numbers of clinical trials of pharmacological interventions are currently being conducted. In this review, we summarize and critically discuss the current and future treatment landscape of IPF, with emphasis on the most promising developmental molecules.

Keywords: Anti-fibrotic agents; Clinical trials; Future perspectives; ILD; IPF; Idiopathic pulmonary fibrosis; Interstitial lung disease; Lung fibrosis; Lung transplantation; Target therapy.

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Conflict of interest statement

Declarations. Conflict of Interest: Elisabetta Cocconcelli declares consulting fees from Boehringer Ingelheim and Chiesi. Elisabetta Balestro declares consulting fees from Boehringer Ingelheim and Roche, speaker fees from Boehringer Ingelheim, and has been a trial investigator for Boehringer Ingelheim and Roche. Paolo Spagnolo reports grants from PPM Services, Roche, Boehringer Ingelheim and Chiesi; consulting fees from PPM Services and Novartis; honoraria from Boehringer Ingelheim; support for attending meetings from PPM Services; participation on a Data Safety Monitoring Board or Advisory Board from AstraZeneca, Trevi, Merck, Galapagos, Novartis and Structure Therapeutics. His wife is an employee of AstraZeneca. Giacomo Giulianelli, Giordano Fiorentù and Nicol Bernardinello have nothing to disclose. Nicol Bernardinello and Paolo Spagnolo are Editorial Board members of Pulmonary Therapy. Nicol Bernardinello and Paolo Spagnolo were not involved in the selection of peer reviewers for the manuscript nor any of the subsequent editorial decisions. Ethical Approval: This article is based on previously conducted studies and does not contain any new studies with human participants or animals performed by any of the authors.

Figures

Fig. 1
Fig. 1
Acute exacerbation of idiopathic pulmonary fibrosis (IPF). Extensive ground glass opacities (stars) superimposed on a background of reticulation, traction bronchiectasis and honeycombing (arrows) in a 75-year-old man
See this image and copyright information in PMC

References

    1. Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19(1):141. - PMC - PubMed
    1. Mei Q, Liu Z, Zuo H, Yang Z, Qu J. Idiopathic pulmonary fibrosis: an update on pathogenesis. Front Pharmacol. 2022. 10.3389/fphar.2021.797292. - PMC - PubMed
    1. Heukels P, Moor CC, von der Thüsen JH, Wijsenbeek MS, Kool M. Inflammation and immunity in IPF pathogenesis and treatment. Respir Med. 2019;147:79–91. - PubMed
    1. Koudstaal T, Wijsenbeek MS. Idiopathic pulmonary fibrosis. Presse Med. 2023;52(3): 104166. - PubMed
    1. Jegal Y, Park JS, Kim SY, Yoo H, Jeong SH, Song JW, et al. Clinical features, diagnosis, management, and outcomes of idiopathic pulmonary fibrosis in Korea: analysis of the Korea IPF Cohort (KICO) Registry. Tuberc Respir Dis (Seoul). 2022;85(2):185–94. - PMC - PubMed

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