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.
Keywords
emphysema - fibrosis - syndrome - pulmonary hypertension - prognosis
