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Review
. 2024 Sep;17(9):817-835.
doi: 10.1080/17512433.2024.2396121. Epub 2024 Aug 27.

Emerging pharmacological options in the treatment of idiopathic pulmonary fibrosis (IPF)

Katyayini Aribindi  1   2 , Gabrielle Y Liu  1 , Timothy E Albertson  1   2
Affiliations
Review

Emerging pharmacological options in the treatment of idiopathic pulmonary fibrosis (IPF)

Katyayini Aribindi et al. Expert Rev Clin Pharmacol. 2024 Sep.

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive-fibrosing lung disease with a median survival of less than 5 years. Currently, two agents, pirfenidone and nintedanib are approved for this disease, and both have been shown to reduce the rate of decline in lung function in patients with IPF. However, both have significant adverse effects and neither completely arrest the decline in lung function.

Areas covered: Thirty experimental agents with unique mechanisms of action that are being evaluated for the treatment of IPF are discussed. These agents work through various mechanisms of action, these include inhibition of transcription nuclear factor k-B on fibroblasts, reduced expression of metalloproteinase 7, the generation of more lysophosphatidic acids, blocking the effects of transforming growth factor ß, and reducing reactive oxygen species as examples of some unique mechanisms of action of these agents.

Expert opinion: New drug development has the potential to expand the treatment options available in the treatment of IPF patients. It is expected that the adverse drug effect profiles will be more favorable than current agents. It is further anticipated that these new agents or combinations of agents will arrest the fibrosis, not just slow the fibrotic process.

Keywords: ACT001; Ao-MMP7; Idiopathic pulmonary fibrosis (IPF); admilparant; bexotegrast; lysophosphatic acids; nintedanib; pirfenidone.

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Conflict of interest statement

TE Albertson has received institutional grants for investigation activities in IPF from Boehringer Ingelheim, Bristol Myers Squibb, and FibroGen. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Figures

Figure 1.
Figure 1.
Most common treatments in IPF for investigational drugs by pathway
Figure 2
Figure 2
(A) Phase development of new therapies by pathways. (B) Phase development of new therapies by agents.
See this image and copyright information in PMC

References

    1. Singh V, Ulasov I, Gupta S, et al. Idiopathic Pulmonary Fibrosis: Where do We Stand and How Far to Go? Discov Med. 2024. Jan;36(180):22–47. - PubMed
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    2. An important paper defining the diagnostic critereon for IPF.

    1. Kropski JA, Blackwell TS, Loyd JE. The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J. 2015. Jun;45(6):1717–27. - PMC - PubMed
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