Combined pulmonary fibrosis and emphysema syndrome: the age of majority

Extract Described in 1990, the combination of pulmonary fibrosis and emphysema is associated with a distinctive physiological profile, with preserved lung volumes and airflow, and severely altered diffusion capacity for carbon monoxide (DLCO) [1]. Combined pulmonary fibrosis and emphysema (CPFE) was individualised as a syndrome in 2005 in a series of 61 patients [2], which drew attention to its main characteristics, namely male predominance, history of smoking, severe dyspnoea on exertion, relatively preserved airflow rates and lung volumes on spirometry, severely impaired DLCO, exertional hypoxaemia, and a high propensity for pulmonary hypertension that occurs in about half of the patients and is associated with dismal prognosis [3]. Identifying CPFE in patients is important owing to the risk of underestimating the severity of the lung disease due to subnormal spirometry. Patients with CPFE have an increased risk of developing lung cancer, even compared to those with emphysema alone or pulmonary fibrosis alone [4]. Although tobacco smoking is by far the main aetiology, CPFE has also been reported in other settings, including connective tissue disease [5], fibrotic hypersensitivity pneumonitis [6], inhalational exposures [7] and rare genetic variants [8, 9].
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Editorials Source Type: research