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Combined pulmonary fibrosis and emphysema
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    Combined pulmonary fibrosis and emphysema

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    Combined pulmonary fibrosis and emphysema
    Other names CPFE
    Specialty Pulmonology

    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.

    Presentation

    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.

    Diagnosis

    The diagnosis is confirmed with high resolution CT scan.

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