Table 4.
Results of functional diagnostic in both sporadic and familial IPF cohorts
| Lung function tests at initial diagnosis | f-IPF (n = 27) | s-IPF (n = 143) | p value |
|---|---|---|---|
| mean value ± SD | mean value ± SD | ||
| Spirometry/ Whole body plethysmography | |||
| VC [% predicted value] | 74 ± 19 | 65 ± 18 | 0.027 |
| FVC [% predicted value] | 75 ± 20 | 62 ± 19 | 0.011 |
| FEV1/ VC [% predicted value] | 104 ± 13 | 108 ± 16 | 0.133 |
| TLC [% predicted value] | 73 ± 21 | 69 ± 18 | 0.293 |
| RV [% predicted value] | 87 ± 41 | 80 ± 30 | 0.547 |
| RV/ TLC [% predicted value] | 97 ± 26 | 108 ± 25 | 0.026 |
| Gas exchange | |||
| DLCO/ SB [% predicted value] | 55 ± 20 | 42 ± 18 | 0.006 |
| Blood gas analysis at rest | |||
| pO2 [mmHg] | 75 ± 13 | 70 ± 10 | 0.015 |
| O2 Saturation [%] | 95 ± 2 | 94 ± 2 | 0.259 |
| pCO2 [mmHg] | 38 ± 3 | 39 ± 6 | 0.262 |
| 6 min-walk test | |||
| SpO2 before test [%] | 95 ± 2 | 95 ± 3 | 0.377 |
| SpO2 after test [%] | 96 ± 2 | 86 ± 8 | 0.0002 |
| 6 MWD [m] | 376 ± 140 | 379 ± 125 | 0.834 |
| O2 Substitution during the test [l/min] | 1 ± 2 | 4 ± 3 | 0.003 |
| Right heart echocardiography | |||
| PAPsys [mmHg] | 32 ± 13 | 40 ± 16 | 0.055 |
Abbreviations: f-IPF familial idiopathic pulmonary fibrosis, s-IPF sporadic idiopathic pulmonary fibrosis, SD standard deviation, VC vital capacity, FVC forced vitality capacity, TLC total lung capacity, RV residual volume, TLCO/sb transfer factor in single breath method, pO2 partial pressure of oxygen, pCO2 partial pressure of carbon dioxide, SpO2 oxygen saturation, VO2max maximum oxygen absorption, PAPsys systolic pulmonal arterial pressure