Combined Pulmonary Fibrosis and Emphysema

The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which an upper lobe emphysema and lower lobe fibrosis coexist in a single patient. These patients have a characteristic lung function profile, with apparently normal or minimally altered dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco), and hypoxemia, which worsens with exercise. Pulmonary hypertension is highly prevalent and is the principal negative prognostic factor for this condition. High resolution computed axial tomography (HRCT) is the main tool to confirm the diagnosis. Cigarette smoking has been proposed as the main factor in its etiology; however, neither pathogenic mechanisms nor the sequence of events involved in this syndrome has been clarified yet. Experimental studies in animal models are providing information on the involvement of some inflammatory mediators in the pathogenesis. There is currently no consensus on the therapeutic approach to be followed in these patients, since the studies published to date on this subject are limited to wellcharacterized series of cases. Therefore, it is a pathology with many unknowns yet to be resolved and highly likely to be underdiagnosed, unless its functional clinical characteristics are taken into account.