# Most Promising Emerging Therapies for Pulmonary Fibrosis: Targeting Novel Pathways

**Authors:** Lorenzo Carriera, Roberto Lipsi, Meridiana Dodaj, Riccardo Inchingolo, Andrea Smargiassi, Angelo Coppola, Pier-Valerio Mari, Roberto Barone, Simone Ielo, Raffaele Scala, Luca Richeldi

PMC · DOI: 10.3390/biomedicines14010154 · Biomedicines · 2026-01-11

## TL;DR

This paper reviews new therapies for pulmonary fibrosis, focusing on drugs targeting novel pathways to improve treatment options and patient outcomes.

## Contribution

The paper highlights emerging pharmacological agents and emphasizes the shift toward precision medicine in treating interstitial lung diseases.

## Key findings

- Nerandomilast has been recently approved, marking progress in antifibrotic therapies.
- Phase III trials are ongoing for new agents with diverse mechanisms of action.
- A precision medicine approach is recommended for better treatment of ILD patients.

## Abstract

Interstitial lung diseases (ILDs) encompass a heterogeneous group of disorders characterized by varying degrees of inflammation and fibrosis. Despite advances in understanding the pathogenesis, therapeutic options remain limited, particularly for patients with progressive phenotypes. Current international guidelines for idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) emphasize the need for antifibrotic strategies and call for novel pharmacological interventions targeting key molecular pathways involved in fibrogenesis. This review provides a comprehensive overview of the most promising emerging pharmacological agents for ILDs, with particular attention to their mechanisms of action, efficacy, and safety profiles as reported in recent preclinical and clinical studies. The recent approval of Nerandomilast and the ongoing phase III trials of other agents mark a pivotal transition toward a new generation of antifibrotic therapies, aiming to achieve more effective disease control and improved patient outcomes. In view of an enlargement of active drugs aiming at controlling the disease with different mechanisms, the Authors underline the need for a “precision medicine” model to be applied to each ILD phenotyped patient, mirroring what already happens for other respiratory diseases.

## Linked entities

- **Chemicals:** Nerandomilast (PubChem CID 166177189)
- **Diseases:** pulmonary fibrosis (MONDO:0002771), idiopathic pulmonary fibrosis (MONDO:0800029)

## Full-text entities

- **Diseases:** inflammation (MESH:D007249), respiratory diseases (MESH:D012140), fibrosis (MESH:D005355), IPF (MESH:D054990), PPF (MESH:D011658), ILD (MESH:D017563)
- **Chemicals:** Nerandomilast (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC12839304/full.md

## References

162 references — full list in the complete paper: https://tomesphere.com/paper/PMC12839304/full.md

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Source: https://tomesphere.com/paper/PMC12839304