PMC8008244_01
Chest X-ray (A) and computed tomography (B-E) at the diagnosis of pleuroparenchymal fibroelastosis showing mild wedge-shaped consolidations in bilateral lung apices (A-C), overinflation of the lower lobes (A), and a flattened chest cage (D) with elevated hilar structures (A). Lower lobes fibrosis was not evident (E).
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Chest X-ray showed the upward shift of hilar structures (Fig. 1A). Chest computed tomography (CT) showed multiple subpleural wedge-shaped airspace consolidations at the apex of both lungs, but the lesions were relatively mild and limited to the upper lobes (Fig. 1B and C). No fibrous lesions were found in the lower lobes (Fig. 1D and E). The thoracic cage was flattened with a flat chest index of 0.50 (reference value in IPF: 0.649 +- 0.006), which was measured as the anteroposterior diameter of the thoracic cage/transverse diameter of the thoracic cage at the level of the 6th thoracic vertebra (Fig. 1D).
Chest X-ray and computed tomography findings from 13 years before the diagnosis (A), 6 years before the diagnosis (B), and 1 year after the diagnosis (C). The hilar structures (arrowheads) on chest radiography were gradually elevated throughout the disease course (B and C). While the wedge-shaped shadows in the bilateral lung apexes did not change significantly, the chest cage gradually became flattened on chest computed tomography (flat chest index of 0.52 in B and 0.49 in C). Pneumothorax in the left lung was evident (arrows). The chest cage was flattened, and the body mass index (BMI) decreased over the course of the disease (D).   indicates death.
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On chest X-ray and CT, the platythorax and elevation of the hilar structures gradually worsened throughout the clinical course of the disease (Fig. 2), while the wedge-shaped shadows in the bilateral lung apexes did not progress significantly (Fig. 2). Even though the lung lesions were mild and did not progress much after the diagnosis, the flat chest index and BMI decreased over time (Fig. 2D).