Related Experiment Video
Updated: Jul 15, 2026
07:51
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
[Combined pulmonary fibrosis and emphysema]
Vincent Cottin1, Pierre-Yves Brillet, Hilario Nunes
1Service de pneumologie, Centre de référence des maladies orphelines pulmonaires, Hôpital Louis Pradel, Lyon, France. vincent.cottin@chu-lyon.fr
Presse Medicale (Paris, France : 1983)
|April 21, 2007
Summary
A newly identified syndrome combines upper-lobe emphysema with lower-lung fibrosis, primarily affecting older male smokers. Smoking is the sole identified risk factor for this condition, characterized by reduced lung function and imaging findings.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- A distinct clinical syndrome characterized by upper-lobe emphysema and lower-lung fibrosis has been recently described.
- This condition predominantly affects male smokers or ex-smokers with a significant smoking history (over 40 pack-years) and a mean age of 65 years.
Purpose of the Study:
- To detail the clinical, functional, and radiological characteristics of this newly identified lung disease.
- To identify key diagnostic features and prognostic factors.
Main Methods:
- Clinical assessment including symptom evaluation (exertional dyspnea) and physical examination (basal crackles).
- Pulmonary function testing assessing respiratory volumes, flows, carbon monoxide transfer, and exercise-induced hypoxemia.
- High-resolution computed tomography (CT) of the chest to evaluate emphysema patterns and fibrotic changes.
Main Results:
- Patients consistently present with exertional dyspnea and basal crackles.
- Pulmonary function tests reveal reduced carbon monoxide transfer and exercise hypoxemia, with often normal or subnormal lung volumes/flows.
- CT findings include centrilobular or upper-zone bullous emphysema, paraseptal emphysema, and diffuse lower-zone fibrosing lung disease (reticular opacities, honeycomb changes, traction bronchiectasis), with increased ground-glass opacities compared to idiopathic pulmonary fibrosis.
- Pulmonary hypertension is observed in nearly half of patients and is a major negative prognostic factor.
Conclusions:
- This syndrome represents a unique clinicoradiological entity strongly associated with smoking.
- Diagnosis relies on characteristic CT findings combined with clinical presentation and pulmonary function abnormalities.
- Pulmonary hypertension significantly worsens the prognosis, with a median survival of approximately 6 years.
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