Combined Pulmonary Fibrosis and Emphysema (CPFE)

Abstract

Emphysema is characterized by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. However, coincidental idiopathic pulmonary fibrosis (IPF) and emphysema was firstly reported in 1990 by Wiggins et al (Wiggins J, et al., 1990) in London. Smoking-related interstitial lung diseases (SRILD) include desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-related interstitial lung disease (RB-ILD), pulmonary Langerhans’ cell histiocytosis (LCH) and idiopathic pulmonary fibrosis (IPF) (Ryu JH, et al., 2001). Tobacco smoking is also major course of emphysema and chronic obstructive pulmonary disease (COPD). Smoking is a common risk factor for both emphysema and pulmonary fibrosis. Recently, the occurrence of both emphysema and pulmonary fibrosis in the same patient has received increased attention as the syndrome of combined pulmonary fibrosis and emphysema (CPFE) (Cottin V, et al., 2005). It has been demonstrated that CPFE syndrome is not rare because on a series of 110 patients with IPF, 28% of them with at least 10% of the lung affected with emphysema, and thus are considered to have CPFE (Mejia M, et al., 2009).