Physiological and radiological findings from published series
| At presentation | During disease course | |
| Pulmonary function 11, 12, 14, 29, 30 | ||
| Normal# | 10 (42) | 4 (97) |
| Obstructive¶ | 29 (42) | 63 (97) |
| Restrictive+ | 26 (42) | 10 (97) |
| Combined obstructive/restrictive | 36 (42) | 15 (97) |
| Low gas transfer§ | 96 (31) | 91 (89) |
| Hypoxaemiaf | 83 (42) | 76 (81) |
| Chest radiograph 2, 13, 14 | ||
| Normal | 5 (147) | 0 (32) |
| Reticulonodular infiltrate | 68 (147) | 94 (32) |
| Cysts/bullae | 47 (147) | 41 (32) |
| Pleural effusion | 5 (78) | 28 (32) |
| Pneumothorax | 35 (78) | 81 (32) |
| Hyperinflation | 27 (147) | 25 (32) |
| Thoracic CT 2, 12, 14 | ||
| Cysts | 100 (104) | 100 (35) |
| Ground-glass opacities | 29 (104) | |
| Nodular densities | 9 (104) | |
| Pneumothorax | 16 (38) | 6 (35) |
| Pleural effusion | 13 (38) | 14 (35) |
| Hilar/mediastinal adenopathy | 6 (104) | |
| Dilated thoracic duct | 11 (35) | |
| Pericardial effusion | 6 (35) | |
| Abdominal CT 20–22 | ||
| Normal | 5 (80) | |
| Renal angiomyolipoma | 53 (111) | |
| Lymphadenopathy | 36 (80) | |
| Lymphangioleiomyoma | 16 (80) | |
| Ascites | 9 (80) | |
| Hepatic angiomyolipoma | 3 (80) |
Data are presented as % (n). CT: computed tomography. #: all values >80% predicted; ¶: forced expiratory volume in one second (FEV1) <80%, FEV1/forced vital capacity (FVC) <70% predicted; +: FVC <80%, FEV1/FVC >70% predicted; §: all values <80% predicted; f: <10.6 kPa.