PMC6143697_01
(A) Chest CT shows reticulation and volume loss in the lower lungs along with severe traction bronchiectasis predominantly in the lower lungs. (B) Panoramic view of a specimen from the left lower lobe shows fibrosing nonspecific interstitial pneumonia with emphysematous change (H&E stain, x 1.25). (C) High-power magnification view of the square in B shows frequent splitting of the secondary alveolar walls (arrow) (H&E stain, x 4); AD: alveolar duct. (D) Magnified view of the circle in B shows concentric intimal fibrosis and medial thickening (Heath-Edward grade 3) of a medium-sized pulmonary artery (arrow) and intimal fibrosis of a pulmonary venule (arrowhead) (Elastica van Gieson stain, x 10). (E) Magnified view of the dotted circle in C shows muscularisation of an arteriole (arrow) of <50 mum in diameter (Elastica van Gieson stain, x 20).
gr1.jpg
PMC6143697_01_gr1.jpg
fig1
Chest computed tomography revealed traction bronchiectasis, reticulation, and ground-glass opacities (Fig. 1A). Surgical lung biopsies revealed a fibrosing pattern indicative of nonspecific interstitial pneumonia (Fig. 1B), prominent splitting of the secondary alveolar walls (i.e., pulmonary emphysema) (Fig. 1C), and pulmonary vasculopathy (Fig. 1D and E).