Related Experiment Video
Updated: Sep 24, 2025
03:38
Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
Published on: June 20, 2025
372
Pirfenidone in Progressive Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
Marya Ghazipura1,2, Manoj J Mammen3, Brittany D Bissell4,5
1ZS Associates, Global Health Economics and Outcomes Research, New York, New York.
Annals of the American Thoracic Society
|May 2, 2022
Summary
Pirfenidone may slow disease progression and protect lung function in patients with progressive pulmonary fibrosis (PPF). However, the evidence quality is very low, requiring cautious interpretation of these antifibrotic treatment benefits.
Area of Science:
- Pulmonology
- Pharmacology
- Clinical Practice Guidelines
Background:
- International societies updated clinical practice guidelines for interstitial lung disease (ILD).
- A systematic review was commissioned to evaluate pirfenidone for progressive pulmonary fibrosis (PPF).
Purpose of the Study:
- To determine the efficacy and safety of pirfenidone in treating patients with progressive pulmonary fibrosis (PPF).
Main Methods:
- Systematic literature search of MEDLINE, EMBASE, and Cochrane databases through December 2020.
- Meta-analysis of data from two included studies on mortality, disease progression, lung function, and adverse events.
- Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach used to assess evidence quality.
Main Results:
- Pirfenidone favored over placebo for forced vital capacity (FVC) and 6-minute-walk distance.
- Pirfenidone showed benefits in diffusing capacity of the lung for carbon monoxide (DLCO) and reduced risk of significant DLCO decline.
- Increased risks of gastrointestinal discomfort and photosensitivity were observed with pirfenidone use.
Conclusions:
- Pirfenidone use is associated with statistically significant decreases in disease progression and protection of lung function in PPF patients.
- The certainty of evidence for these effects is very low due to study limitations.
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