Combined pulmonary fibrosis and emphysema
|Combined pulmonary fibrosis and emphysema|
|Other names: CPFE|
|Chest X-ray of combined pulmonary fibrosis and emphysema demonstrating bilateral interstitial pattern, predominantly right sided|
Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema. The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor.
Signs and symptoms
CFPE is characterised by shortness of breath, and reduced oxygen concentration (reflecting gas exchange abnormalities). Imaging shows upper-lobe emphysema, and lower-lobe interstitial fibrosis. CFPE is often complicated by pulmonary hypertension, acute lung injury, lung cancer, and coronary artery disease.
The primary risk factor for Combined pulmonary fibrosis and emphysema is smoking
In terms of management there is no specific treatment, though it is important to stop smoking .Some individuals might benefit from ICS/LABA treatment
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