Medical Science: Updates and Prospects Vol. 10

Combined pulmonary fibrosis and emphysema (CPFE) is a rare but increasingly recognised condition characterised by the simultaneous coexistence of both upper lobe predominant emphysema and diffuse pulmonary fibrosis mainly in the lower lobe. The exact pathogenetic mechanisms underlying the development of CPFE remain unclear. The condition is most commonly observed in current or former heavy smokers. In patients with CPFE, spirometry findings may be normal or show only mild abnormalities. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and total lung capacity (TLC) are usually within normal limits or only slightly reduced. High-Resolution Computed Tomography (HRCT) has a pivotal role in diagnosis. Pulmonary function test showed relatively preserved lung volumes and reduced diffusing capacity of the lung for carbon monoxide (DLCO). Development of pulmonary hypertension (PH) is largely attributed to morbidity in patients with CPFE, which is the principal prognostic factor for this condition. Despite growing recognition, the natural history and optimal management strategies for CPFE are not well established. This review summarises current knowledge on the pathogenesis, clinical characteristics, management, and prognostic factors of CPFE. Future studies are required to ascertain the aetiology, morbidity, mortality and management of CPFE, with or without PH, and also to delineate more precisely the boundaries between IPF and patients with CPFE syndrome.