Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease

Abstract

Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DL(co)) and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.

Figure 1

Chest X-ray of a patient diagnosed with combined pulmonary fibrosis and emphysema demonstrating bilateral interstitial pattern, predominantly right sided, with reticulonodular infiltrates in the lung bases and subpleural region, and a reduction of lung density in upper lung fields, mainly on the left.

Figure 2

High-resolution computerized tomography (HRCT) of the same patient. (a) Presence of paraseptal emphysema and subpleural bullae (white arrowheads) and centrilobular emphysema (arrows) in both upper lobes. (b) Reticular interstitial disease with intralobular thickening and images of subpleural honeycombing and traction bronchiectasis (black arrowheads), (c) Reticular interstitial disease in middle and right lower lobes, with interlobular septal thickening, subpleural honeycombing, and traction bronchiectasis. (d) Coronal reconstruction in the posterior regions of both lungs: bilateral paraseptal emphysema (white arrowheads) and reticular interstitial disease and honeycombing in right lower lobe.