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Updated: Dec 24, 2025
07:51
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
796
Combined Pulmonary Fibrosis and Emphysema
Maria Bolaki1, Katerina M Antoniou1
1Department of Respiratory Medicine, Heraklion University Hospital, University of Crete School of Medicine, Heraklion, Crete, Greece.
Seminars in Respiratory and Critical Care Medicine
|April 13, 2020
Summary
Combined pulmonary fibrosis and emphysema (CPFE) presents unique lung function challenges. Understanding its unclear causes, complications like pulmonary hypertension, and treatment is crucial for patient prognosis.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Combined pulmonary fibrosis and emphysema (CPFE) involves upper lobe emphysema and lower lobe fibrosis from interstitial lung diseases.
- Patients exhibit preserved lung volumes but reduced carbon monoxide transfer.
- The pathogenesis of coexisting emphysema and fibrosis is not well understood.
Purpose of the Study:
- To review current knowledge on CPFE, covering epidemiology, pathogenesis, clinical and radiologic features.
- To discuss complications, prognosis, and potential management strategies for CPFE.
- To highlight CPFE as a distinct manifestation in connective tissue disease-related lung issues.
Main Methods:
- Literature review of existing research on CPFE.
- Synthesis of data on epidemiology, pathogenesis, and clinical presentation.
- Analysis of radiologic findings, complications, and prognostic factors.
Main Results:
- CPFE is characterized by specific lung function profiles and is associated with significant complications.
- Pathogenic mechanisms remain unclear, with various theories proposed.
- No established management guidelines currently exist for CPFE.
Conclusions:
- CPFE is a complex syndrome with poor prognosis, often complicated by pulmonary hypertension, exacerbations, and cancer.
- Further research into pathogenesis and treatment is needed.
- CPFE requires a multidisciplinary approach to management.
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