CTICasesCombined Pulmonary Fibrosis and Emphysema (CPFE) in a Never-Smoker
Imaging findings
CT chest demonstrates a combination of mild, upper-lobe-predominant paraseptal emphysema and a subpleural, basal-predominant UIP pattern of lung fibrosis with traction bronchiectasis. Over seven years of follow-up, there is significant progression of the subpleural fibrosis and traction bronchiectasis, alongside the development of marked tracheal dilation and corrugation resembling Mounier-Kuhn syndrome.
Key takeaways
Combined pulmonary fibrosis and emphysema is strongly associated with a history of heavy smoking. Finding both paraseptal emphysema and progressive UIP-like fibrosis in a lifetime never-smoker is highly unusual and should prompt a thorough evaluation for occult connective tissue diseases (such as rheumatoid arthritis) or alternate chronic exposures. Additionally, the progression of progressive lung fibrosis can be associated with tracheobronchomegaly and expiratory airway collapse.
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