PMC7567044_01
Imaging studies. (A) A chest X-ray at the time of the first visit showing enlargement of the mediastinum. (B) Chest CT with the lung window at the time of the first visit showing the infiltrations around bilateral hilar areas. (C) Chest CT with the soft-tissue window at the time of the first visit showing the enlarged soft-tissue infiltration in the mediastinum. (D) Thoracic MRI at the time of the first visit showing the T2 high-intensity dilated soft tissue in the mediastinum and bilateral hilar areas. (E) Chest CT with the lung window on admission showing ground-glass opacities in addition to the infiltrations already confirmed on the previous CT. (F) CT taken 24 hours after intranodal lymphangiography. Lipiodol accumulation is confirmed in the mediastinum and bilateral hilar areas.
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When he first came to our hospital, the chest X-ray showed bilateral infiltration, in addition to dilation of the mediastinum (Fig. 1A). Chest CT showed abnormally enlarged soft tissue surrounding the mediastinum and pericardial area, as well as peribronchovascular and interstitial septal thickening (Fig. 1B and C). Thoracic magnetic resonance imaging (MRI) showed T2 high-intensity dilated soft tissue in the mediastinum and bilateral hilar areas (Fig. 1D). Chest CT showed ground-glass opacities, reflecting the current alveolar hemorrhage, in addition to enlargement of the mediastinum and the interstitial septal thickening that had already been seen on previous CT scans (Fig. 1E). CT obtained after lymphangiography with lipiodol showed accumulation of lipiodol in the dilated mediastinal soft tissue, pericardium, and bilateral hilar areas (Fig. 1F).
Gross findings. (A) Bronchoscopic findings. A view of the right bronchus intermedius showing a diffuse erythematous mucosa and clots. RML, right middle lobe; RLL, right lower lobe. (B) Thoracoscopic findings of pleural surface showing yellowish pleura with diffuse telangiectasia. LLL, left lower lobe.
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On bronchoscopy, diffuse mucosal erythematous change and uncountable clots were reported (Fig. 2A). Thoracoscopy showed yellowish pleura with diffuse telangiectasia (Fig. 2B).
Histopathological findings of the lung. (A) Elastica van Gieson staining, x12.5, showing dilated venous vessels in the subpleural area (arrow). (B) (C) D2-40, magnification x100, showing dilated lymphatic vessels in the subpleural area (arrows). (D) Elastica van Gieson staining, magnification x100, showing irregularity in vascular wall thickness and a vulnerable area leading to alveoli (arrow). (E) Prussian blue staining, magnification x200, showing hemosiderin-laden alveolar macrophages (arrowheads) around dilated veins (arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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Histologically, the SLB samples taken from segments 6 and 10 of the left lung showed dilated lymphatic vessels and veins in the subpleural area and interlobular septa (Fig. 3A-C). Dilated veins showed heterogeneity in wall thickness and a vulnerable area leading to alveoli (Fig. 3D). Prussian blue staining showed hemosiderin-laden macrophages around these dilated vessels, suggesting past alveolar hemorrhage (Fig. 3E).