PMC6010663_01
Inspiratory high resolution computed tomography (HRCT) images showing upper lobe predominant cysts. There is no traction bronchiectasis or reticulation.
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High resolution computed tomography (HRCT) of the lungs showed cystic changes and non-specific discrete fibrosis with an upper lobe predominance (Fig. 1).
2a: Honeycombing fibrosis, lymphocytic inflammation and macrophages in the alveoli (hematoxylin-eosin, 100x).2b: Interstitial fibrosis and alveolar macrophages positive for CD68 (40x). 2c: Heterogeneous fibrosis with smooth muscle hyperplasia (smooth muscle actin, 40x).
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Fig. 2 shows the histology of the patient's lung biopsy from lingula in the left lung.
3a: Inspiratory HRCT images of upper lobe showing progression of cysts. 3b: Inspiratory HRCT images at the level of carina showing progression of cysts, tractions bronchiectasis and fine reticulation. 3c: Inspiratory HRCT images of lower lobes showing honeycombing (progression of the cysts in multiple layers).
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A new HRCT showed progressive cystic changes in the upper lobes, traction bronchiectasis and ground glass opacities (GGO) (Fig. 3a, b and 3c).
4a: Inspiratory HRCT images of the upper lobe showing cysts and fine reticulation.4b: Inspiratory HRCT images of the lower lobe showing fine reticulation, cysts and traction bronchiectasis.
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Family tree.
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The patient's family tree is shown in Fig. 5.