Fig. 3

Patient 2. Radiological evolution. Axial CT images of the lung. a CT scan at first presentation (34 years old) demonstrates a subtle ground glass infiltrate, smooth interlobular septal thickening and a focal nodule in the subpleural left lower lobe. b At 40 years old, imaging reveals a more obvious ground glass infiltrate. c At 42 years old, perivascular emphysema/cysts are evident (white arrow), which d subsequently progresses to emphysema 2 years later. Similarly to Fig. 1 d this has a perivascular distribution