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Expert Rev Respir Med. 2013 Feb;7(1):19-31; quiz 32. doi: 10.1586/ers.12.80.

Combined pulmonary fibrosis and emphysema.

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Second Pulmonary Medicine Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece.


The advent of computed tomography permitted recognition of the coexistence of pulmonary fibrosis and emphysema (CPFE). Emphysema is usually encountered in the upper lobes preceding fibrosis of the lower lobes, and patients are smokers, predominantly male, with distinct physiologic profile characterized by preserved lung volumes and markedly reduced diffusion capacity. Actually, the term CPFE is reserved for the coexistence of any type and grade of radiological pulmonary emphysema and the idiopathic usual interstitial pneumonia computed tomography pattern as well as any pathologically confirmed case. CPFE is complicated by pulmonary hypertension, lung cancer and acute lung injury and may present different outcome than that of its components.

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