Combined pulmonary fibrosis and emphysema

Abstract

Purpose of review: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized by upper lobe emphysema with lower lobe fibrosis. We aim to bring some clarity about its definition, nature, pathophysiology, and clinical implications.

Recent findings: Although multiple genetic and molecular pathways have been implicated in the development of CPFE, smoking is considered the most prevalent risk factor. CPFE is most prevalent in middle-aged men with more than 40 pack-years of smoking and can be seen in about 8% of all chronic obstructive pulmonary disease (COPD) patients. Given its nature, it is a radiological diagnosis, better defined by computed tomography (CT). Spirometry can be normal despite severe disease or can have restrictive or obstructive patterns, but the diffusing capacity of the lungs (DLCO) is consistently low regardless of the spirometry pattern. The disease is progressive, with high occurrences of lung cancer and pulmonary hypertension, complications that limit survival. Unfortunately, there is no treatment found to be beneficial other than supportive care and guideline-directed medical therapy.

Summary: CPFE is best described as a clinical and radiological syndrome where smokers are particularly at greater risk. Although simplistic, the earliest definition based chiefly on radiographic findings can identify a patient population with similar physiology. The most recent consensus proposes the definition based on mainly radiological findings with impaired gas exchange.