CPFE - A New Entity with a Diagnostic Dilemma

Md. Ashik Imran Khan1, Mohammed Kamrul Hasan2,ASM Fateh Akram3, AHM Raihanuzzaman,Sarker3, Salma Akhter4, Md. Khairul Hassan Jessy5

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 (DLCO) 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. Here we report a case of a patient with both fibrosis and emphysema who fit
partial criteria of diagnosis of CPFE and put us in a diagnostic dilemma.
Keywords: Pulmonary fibrosis, Emphysema, Pulmonary function test, High resolution computed tomography
(HRCT), pulmonary hypertension (PH).

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