Pulmonary function tests at presentation
| Patient | TLC % pred | VC % pred | FEV1 % pred | FEV1 % pred/FVC % | DL,CO % pred | KCO % pred | Pa,O2 mmHg | Pa,CO2 mmHg |
| 1 | 63 | 52 | 58 | 90 | 76 | 102 | 78 | 41 |
| 2 | 76 | 73 | 80 | 84 | 49 | 67 | 64 | 36 |
| 3 | 70 | 50 | 52 | 82 | 26 | 55 | 54 | 35 |
| 4 | 71 | 67 | 65 | 82 | 31 | 42 | 78 | 36 |
| 5 | 41 | 34 | 40 | 91 | 32 | 84 | 70 | 37 |
| 6 | 73 | 85 | 92 | 86 | 50 | 79 | 69 | 37 |
| 7 | 62 | 58 | 54 | 78 | 36 | 42 | 70 | 35 |
| 8 | 65 | 64 | 65 | 88 | 31 | 52 | 66 | 42 |
TLC: total lung capacity; % pred: % predicted; VC: slow vital capacity; FEV1: forced expiratory volume in one second; DL,CO: diffusing capacity of the lung for carbon monoxide; KCO: transfer coefficient of the lung for carbon monoxide; Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension.