Mortality in combined pulmonary fibrosis and emphysema patients is determined by the sum of pulmonary fibrosis and emphysema

Abstract

Emphysema is one of the most common pulmonary comorbidities of idiopathic pulmonary fibrosis (IPF), presenting in about one-third of IPF patients [1]. The term combined pulmonary fibrosis and emphysema (CPFE) has been used to describe a potential phenotype characterised by the coexistence of upper lobe-predominant emphysema, lower lobe-predominant fibrosis and relative preservation of lung volumes (forced vital capacity; FVC) in the context of a disproportionately reduced gas transfer (diffusing capacity of the lung for carbon monoxide; DLCO) [1–3]. With regard to patient survival, it remains unclear whether mortality in patients with CPFE reflects the cumulative effects of two disease processes (emphysema and fibrosis), or whether CPFE represents a distinct disease phenotype where outcome is worse than the sum of disease parts (emphysema and fibrosis).