Related Experiment Video
Updated: Jun 13, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Idiopathic pulmonary fibrosis.
Joseph P Lynch1, Michael C Fishbein, Rajeev Saggar
1The David Geffen School of Medicine at UCLA, Division of Pulmonary, Critical Care Medicine, and Hospitalists, 10833 Le Conte Avenue, Room CHS 37-131, Los Angeles, CA 90095, USA. jplynch@mednet.ucla.edu
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with unknown causes, characterized by usual interstitial pneumonia (UIP). Current treatments are unproven, and lung transplantation is an option for eligible patients.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia of unknown etiology.
- It is histologically defined by the usual interstitial pneumonia (UIP) pattern.
- UIP can be diagnosed via surgical lung biopsy or high-resolution CT scans.
Purpose of the Study:
- To clarify the definition and diagnostic criteria for IPF.
- To elucidate the pathogenesis and prognosis of IPF.
- To review current therapeutic options for IPF.
Main Methods:
- Review of existing literature on IPF and UIP.
- Analysis of diagnostic modalities including histology and imaging.
- Evaluation of pathogenetic mechanisms and clinical outcomes.
Main Results:
- IPF should be exclusively defined as idiopathic UIP.
- Alveolar epithelial cell injury and fibroblast alterations are key pathogenetic elements.
- The prognosis for idiopathic UIP is poor, with a median survival of approximately 3 years.
Conclusions:
- Accurate diagnosis and classification of IPF are crucial.
- Current medical therapies for IPF have unproven efficacy.
- Lung transplantation remains a viable treatment for advanced disease.
Related Concept Videos
Chronic Obstructive Pulmonary Disease I: Introduction
Chronic Obstructive Pulmonary Disease II: Emphysema
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Inflammation
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features
Chronic Obstructive Pulmonary Disease-I: Introduction
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...