Combined pulmonary fibrosis and emphysema (CPFE) is a clinical syndrome characterized
by upper lobe emphysema and lower lobe fibrosis manifested by exercise hypoxemia,
normal lung volumes, and severe reduction of diffusion capacity of carbon monoxide.
It has varying prevalence worldwide with a male predominance, and with smoking history
of more than 40 pack-years being a common risk factor. The unique imaging features
of CPFE emphasize its distinct entity, aiding in the timely detection of pulmonary
hypertension and lung cancer, both of which are common complications. High-resolution
computed tomography (HRCT) is an important diagnostic and prognostic tool, while lung
cancer is an independent factor that alters the prognosis in CPFE patients. Treatment
options for CPFE are limited, but smoking cessation, usual treatments of pulmonary
fibrosis and emphysema, and avoidance of environmental exposures are encouraged.
