Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias

Figure 1

Case  1. (a) Chest radiography shows bilateral pulmonary involvement, with reticular interstitial pattern, with marked predominance in the left lung, more prominent on the left and signs of volume loss of both upper lobes with bilateral pleural thickening of apical predominance. (b) Axial high resolution computed tomography (HRCT) scan obtained through apical region shows pleural thickening, much more marked in left apex. (c) Axial HRCT scan at level of carina reveals right side marked pleural thickening and signs of pulmonary fibrosis, mainly in the left upper lobe, with the presence of multiple traction bronchiectasis, superimposed with ground-glass opacities. (d) Coronal HRCT reformatted image shows left apical and lateral right pleural thickening. Note architectural distortion reflecting fibrotic changes, accompanied by traction bronchiectasis and scattered peripheral reticular opacities.

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