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