Combined Pulmonary Fibrosis and Emphysema

Maria Bolaki, MD, PhD; Katerina M. Antoniou, MD, PhD

Disclosures

Semin Respir Crit Care Med. 2020;41(2):177-183. 

In This Article

Abstract and Introduction

Abstract

Combined pulmonary fibrosis and emphysema (CPFE) is a clinical entity characterized by the combination of upper lobe emphysema and lower lobe fibrosis, the latter owing to various interstitial lung diseases. These patients have a characteristic lung function profile, with relatively preserved dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer. The pathogenic mechanisms leading to the coexistence of emphysema with fibrosis remain unclear and different theories have been proposed. CPFE is frequently complicated by pulmonary hypertension, acute exacerbations, and lung cancer leading to poor natural history and prognosis. The syndrome of CPFE represents a distinct pulmonary manifestation in the spectrum of lung diseases associated with connective tissue diseases. Currently, there are no established recommendations regarding the management of patients with CPFE. We provide a review on the existing knowledge of CPFE regarding the epidemiology, pathogenesis, clinical manifestations, radiologic appearance, complications, prognosis, and possible treatment options.

Introduction

Emphysema and idiopathic pulmonary fibrosis (IPF) are two distinct entities: emphysema is characterized by enlargement of airspaces, distal to terminal bronchioles due to destruction of the surrounding tissues,[1] whereas IPF belongs to the idiopathic interstitial lung diseases (ILDs) and is characterized by the usual interstitial pneumonia (UIP) pattern on histopathology.[2] In 1974 Auerbach et al first described the coexistence of these two entities in autopsy specimens of smokers.[3] In 1990, Wiggins et al reported eight patients with both pulmonary emphysema and fibrosis who had a significant reduction in gas transfer.[4] Seven years later, a study quantified the different effects of ILD and emphysema on pulmonary function.[5] In 2005, for the first time Cottin et al characterized this condition as a well-defined syndrome termed "combined pulmonary fibrosis and emphysema" (CPFE).[6] As there is increasing interest in this new entity in the literature, in this article we will review the particular features of CPFE and we will also discuss some of the most recent evidence published.

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