PMC7371979_01
HRCT images showing diffuse pleural and subpleural thickening with consolidations, architectural distortion and traction bronchiectasis accompanied by volume loss in the upper lobes (A), also in the apical segment of lower lobes (B).
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High-resolution CT (HRCT) of the chest demonstrated subpleural thickening, volume loss and architectural distortion predominantly found in both lung apices and reticulation in basal region, consistent with PPFE (Fig. 1A, B).
Lung biopsy specimen (H&E stain, x100) and ((EvG) stain X100) at low power:. (A) Demonstrated the peripheral lung parenchyma with a transition of extensive elastosis with fibrosis.. (B) Shows bronchiolus with metaplastic hyperplasia of smooth muscle mixed with dense elastosis and fibrosis.. (C) EVG staining demonstrates elastosis and fibrosis.. (D) EVG staining demonstrates extensive elastosis in the submucosa.
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The pathological specimen demonstrated tissue with interstitial fibro-elastosis, consistent with PPFE (Fig. 2A-D).
PMC7371979_02
High-resolution CT showed symmetrical pleural and subpleural fibrosis at the periphery of the upper lobes (A). Also a patch of fibrosis and traction bronchiectasis in the lingula with additional foci of subpleural scarring in both lower lobes (B).
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A). A larger patch of fibrosis with marked traction bronchiectasis was present in the lingula with additional foci of subpleural scarring in both lower lobes (Fig. 3 B).