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Combined Pulmonary Fibrosis and Emphysema with Spontaneous Pneumothorax

Imaging findings

CT chest shows upper-lobe centrilobular emphysema combined with basal-predominant, subpleural reticulation, traction bronchiectasis, and honeycombing (UIP pattern). A spontaneous left-sided hydropneumothorax is present, with dynamic expiratory CT demonstrating preferential collapse of the left upper lobe.

Key takeaways

Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity characterized by the co-existence of upper-lobe emphysema and lower-lobe fibrosis. Patients are at high risk for spontaneous pneumothorax. On dynamic expiratory scans, the emphysematous upper lobes collapse preferentially due to loss of elastic recoil, while the fibrotic lower lobes remain relatively rigid.

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