Fact-sheet: Combined pulmonary fibrosis emphysema syndrome
Updated on 04/03/2018 at 9:02 AM
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Definition
Combination on imaging of centrilobular and/or paraseptal emphysematous lesions and fibrotic changes.
Belongs to the category of smoking-related interstitial lung diseases
Clinical features
- Current or former smokers
- Male predominance
- Age at diagnosis: 60-70 years
- Severe, disabling dyspnea
- May occur in the setting of systemic sclerosis and rheumatoid arthritis
- Genetic forms currently being identified
- PFTs: preserved lung volumes (VC averaging 90%) but impaired CO transfer (DLCO averaging 37%)
Radiography
- Apical hyperlucency
- Basal interstitial pattern
CT
At the apices:
- Centrilobular emphysema: hypodense area without a visible wall, occupying the center of the secondary lobule, centered on a vessel
- Paraseptal emphysema: distended hypodense secondary lobules, separated by interlobular septa, located in the subpleural regions.
At the bases: signs of fibrosis
- subpleural intralobular reticulation
- honeycombing
- +/- traction bronchiectasis
Management
Mean survival: 6.1 years
Complications:
- Lung cancer
- Pulmonary hypertension
- Chronic respiratory failure
- Exacerbations
Treatment: smoking cessation, symptomatic treatments, and sometimes antifibrotics
Classification
Topographic patterns:
- Gradual transition: diffuse emphysema with progressive decrease in bulla size from the apices to the lung bases, with a transition zone where emphysema and coarse honeycombing can no longer be distinguished.
- Paraseptal emphysema: predominance of paraseptal emphysema, with confluent bullae at the bases with visible walls and associated fibrosis.
- Separate entities: fibrosis and emphysema appear topographically unrelated.
Differential diagnosis