Related Experiment Video
Updated: Jun 13, 2026
03:38
Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
Published on: June 20, 2025
Progressive pulmonary fibrosis: a state-of-the-art review
Vincent Cottin1,2, Kevin Brown3, Kevin R Flaherty4
1National Reference Centre for Rare Pulmonary Diseases and Constitutive Centre for Pulmonary Hypertension, Louis Pradel Hospital, Hospices Civils de Lyon, ERN-LUNG, Lyon, France.
The European Respiratory Journal
|June 11, 2026
Summary
Progressive pulmonary fibrosis (PPF) is a serious lung condition. New treatments like nerandomilast show promise in slowing disease progression, offering hope for patients with fibrosing interstitial lung diseases.
Area of Science:
- Pulmonology
- Fibrotic Interstitial Lung Diseases
- Progressive Pulmonary Fibrosis
Background:
- Fibrosing interstitial lung diseases (ILDs), including idiopathic pulmonary fibrosis (IPF), lead to irreversible lung function loss and mortality.
- A significant subset of patients with fibrosing ILDs develop a progressive phenotype (PPF) similar to IPF, unresponsive to conventional treatments.
- PPF is characterized by worsening respiratory symptoms, declining lung function, reduced quality of life, and premature death.
Purpose of the Study:
- To review the concept and management of progressive pulmonary fibrosis (PPF).
- To highlight recent therapeutic advancements in treating PPF.
- To emphasize the importance of early PPF diagnosis for timely intervention.
Main Methods:
- Review of clinical trials and practice guidelines for fibrosing ILDs and PPF.
- Analysis of data on antifibrotic therapies including nintedanib, nerandomilast, and pirfenidone.
- Synthesis of evidence regarding disease progression markers and treatment efficacy.
Main Results:
- Nintedanib demonstrated a significant reduction in disease progression in PPF patients.
- Nerandomilast further reduced disease progression in PPF.
- Pirfenidone has shown potential benefits in smaller trials for PPF.
Conclusions:
- Early diagnosis of PPF is crucial for initiating antifibrotic therapy and improving patient outcomes.
- Nintedanib and nerandomilast are key treatments for managing PPF progression.
- Ongoing research is developing novel compounds for IPF and PPF treatment.
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