Table 4.

Imaging Characteristics of Pleuroparenchymal Fibroelastosis

Chest Radiograph Abnormally narrowed anterior-posterior thoracic dimension (flattened thoracic cage) Elevated hilar opacities Reticular and nodular opacities in the bilateral upper lung fields Fibrocystic opacities in the upper lung fields and occasional reticular opacities in the lower lung fields in the advanced stage Chest CT Initial stage: Subpleural nodular and reticular opacities in the apex, but minimal changes in the middle and lower lobes Advanced stage: Fibrotic opacities with traction bronchiectasis extending to adjacent lobes with multiple bulla and large cysts at the upper lung fields Occasional subpleural reticular opacities in the bilateral lower lobes resembling usual interstitial pneumonia (UIP)