Diagnosis and treatment of Combined Pulmonary Fibrosis and Emphysema (CPFE)

Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combination pulmonary fibrosis and emphysema which is now widely found. Clinical characteristics of the CPFE are current or former heavy smoker, predominantly male, and symptom of shortness of breath on exertion. The standard diagnosis of CPFE is based on high resolution computed tomography (HRCT) with emphysema in the upper lung zone and fibrosis in the lower lung zone. The lung function profile in CPFE is different from patients with emphysema or pulmonary fibrosis alone. The lung volumes in CPFE is preserved, but there is significant reduction in lung diffusion capacity. CPFE has a poor prognosis. The presence of pulmonary hypertension in CPFE is a poor prognostic factor. Because there is no current standard treatment of CPFE, the focus of management is supportive therapy, management of complications, and management of exacerbation CPFE.