Combined pulmonary fibrosis and emphysema with pulmonary arterial hypertension: A Rare Case Report.

Combined pulmonary fi brosis and emphysema (CPFE) is a recently recognized radiologically defi ned syndrome characterized by simultaneous coexistence of both upper lobe emphysema and lower lobe pulmonary fi brosis. We present a case of CPFE in a 57-year-old male smoker who presented with dry cough, progressive breathlessness, and swelling of feet. His chest X-ray revealed bilateral lower Zone reticulonodular opacities with hyperlucent upper Zones. Pulmonary function tests showed wellpreserved lung volumes and reduced diff using capacity of the lung for carbon monoxide. High-resolution computed tomogram showed bilateral lower lobe subpleural honeycombing along with fi brosis and traction bronchiectasis with bilateral upper lobe emphysema predominantly paraseptal type. His 2D echo was suggestive of moderate pulmonary arterial hypertension.