PMC5384413_01
High-resolution computed tomography of the chest with major diffuse centrilobular emphysema, characteristic signs of pulmonary veno-occlusive disease and no evidence of pulmonary fibrosis: patchy ground-glass opacities, and few septal lines; and mediastinal and hilar lymphadenopathies. Note the presence of significant development of arteries in the mediastinum from the systemic circulation.
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High-resolution CT of the chest showed major diffuse centrilobular emphysema, patchy ground-glass opacities, few septal lines, and mediastinal and hilar lymphadenopathies (Fig. 1).
Lung histology of samples obtained after lung transplantation. As typically seen in pulmonary veno-occlusive disease (PVOD), small pulmonary veins and venules are partially or completely occluded by paucicellular fibrous thickening of the intimal layer (a). In addition, pulmonary arteries (b, bottom left) display eccentric wall-thickening. Also, focal broadening of the alveolar walls with multiplication of alveolar capillaries is observed (b, inset center), corresponding to pulmonary capillary hemangiomatosis, a feature that is typically observed in PVOD, these patchy foci are frequently associated with muscularized microvessels (b, inset top). Note the areas with emphysematous loss of alveoli (b, top and bottom).
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Histology of the explanted lungs showed pre- and post-capillary lesions involving pulmonary arteries, pulmonary veins, and pre-septal venules, leading to partial occlusion of the lumen (Fig. 2).