CTICasesIdiopathic Pleuroparenchymal Fibroelastosis (PPFE) with Mixed NSIP/Organizing Pneumonia Features
Imaging findings
A woman undergoing pulmonary fibrosis transplant evaluation had CT showing extensive bi-apical, upper-zone-predominant, peripheral dense fibrotic tissue with traction bronchiectasis and marked upper lobe volume loss, an appearance likened to an upside-down UIP distribution. Lower down, the disease was more peribronchial and subpleural with confluent opacity suggesting a fibrosing organizing pneumonia or NSIP-spectrum process, without the typical distribution of usual interstitial pneumonia, hypersensitivity pneumonitis, or a clear connective tissue disease pattern.
Key takeaways
Marked upper-lobe volume loss with dense pleural-based fibrosis and traction bronchiectasis, especially when combined with a more NSIP- or organizing-pneumonia-like pattern in the lower lobes, is characteristic of pleuroparenchymal fibroelastosis (PPFE), which can occur in isolation or overlap with UIP or other fibrosing patterns both pathologically and on imaging; PPFE is one of the rarer entities in the current ATS/ERS idiopathic interstitial pneumonia classification, and familial pulmonary fibrosis (including telomere-related disease, which can produce strikingly different imaging patterns even among affected relatives) should be considered when the pattern is unusual.
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