Combined pulmonary fibrosis and emphysema: bad and ugly all the same?

Emphysema is highly prevalent in patients with idiopathic pulmonary fibrosis (IPF) [1] and interstitial lung disease (ILD) associated with rheumatoid arthritis [2], conditions linked to tobacco smoking [3]. The combination of pulmonary fibrosis and emphysema (CPFE) has been suggested to be a syndrome [4, 5], based on distinctive clinical, radiological, functional and outcome features [6]. Indeed, patients with CPFE reproducibly present with a distinct functional profile consisting of relative preservation of lung volumes and greater reduction in diffusing capacity of the lung for carbon monoxide (DLCO) [4]. In a pioneering study, WELLS et al. [7] showed that patients with IPF and emphysema had higher lung volumes by 5–10% and lower DLCO by ∼15% as compared to subjects with IPF alone, after adjustment for the extent of ILD.