Table 3.
Imaging findings
| PPFE severity (last control) | ILD pattern in other zones | Progressive thorax cage flattening | Air trapping (exp) | PAH (mm) | Asbestos-related calcified pleural plaques | Other features | Imaging of the native lung | 18F-FDG PET/CT | Evolution | |
|---|---|---|---|---|---|---|---|---|---|---|
| Lung transplant | ||||||||||
| Case 1 | 1 | 0 | No | - | No | No | Apical paraseptal emphysema | Lung tumor and UIP | Uptake of the pleuroparenchymal opacities | Slow |
| Case 2 | 3 | 0 | No | - | No | No | Tracheobronchial diverticula | UIP | - | Rapid |
| Case 3 | 2 | 0 | No | Right middle lobe air trapping (bronchomalacia) | Pulmonary trunk: 32; RPA: 29; LPA: 28 | No | Apical paraseptal emphysema, left superior lobar bronchus stenosis with apical atelectasia. | Pulmonary hypertension (Eisenmenger’s syndrome) | Uptake of the pleuroparenchymal opacities | Rapid |
| Case 4 | R: 3 L: 1 |
0 | No | Peripheral air trapping and tracheomalacia | Pulmonary trunk: 39; RPA: 25; LPA: 26 | No | Bilateral upper lobes bronchovascular cysts | Postembolic pulmonary hypertension | - | Slow |
| HSCT | ||||||||||
| Case 5 | R: 4 L: 1 |
0 | No | No | Pulmonary trunk: 32; RPA: 21; LPA: 20 | No | No | X | - | Slow |
| Case 6 | R: 2 L: 3 |
0 | Yes | - | Pulmonary trunk: 3; RPA: 25; LPA: 25 | No | Left pulmonary artery trombosis; “tree in bud” in the lower left lobe; thin anterior pneumothorax | X | Uptake of the pleuroparenchymal opacities | Slow |
PPFE, pleuroparenchymal fibroelastosis; ILD, interstitial lung disease; exp, expiratory CT; PAH, pulmonary arterial hypertension; 18F-FDG PET/CT: fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography; UIP, usual interstitial pneumonia; RPA, right pulmonary artery; LPA, left pulmonary artery; L, left lung; R, right lung; HSCT, hematopoietic stem cell transplantation.