Fig. 5

Patient 3. Radiologic evolution. Axial CT images of the lungs; a: At 9 years old the patient presented with an acute flare, post-contrast CT demonstrates ground glass nodules which resolved on follow up chest radiographs (not shown); b: At 11 years old a cavitating pulmonary nodule is present in the right lower lobe, and emphysema has developed surrounding the pulmonary vessels (arrow). The pulmonary nodule resolved on interval CT (not shown); c: At 15 years old the emphysema has proliferated, with a perivascular component again present. An acute ground glass infiltrate is also present; d: At 20 years old, peripheral polygonal areas of consolidation are present within the peripheral pulmonary parenchyma/pleura, in keeping with pleuroparenchymal fibroelastosis. A ground glass infiltrate is also present, as is emphysema