Combined pulmonary fibrosis and emphysema

Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema.^[1][2] The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor.^[3]

Combined pulmonary fibrosis and emphysema
Other names	CPFE
Specialty	Pulmonology

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.^[4] CFPE is often complicated by pulmonary hypertension, acute lung injury, lung cancer, and coronary artery disease.^[4]

Diagnosis

The diagnosis is confirmed with high resolution CT scan.^[5]