Table 1.
FPF | HPSPF | URel | p-Value | |
---|---|---|---|---|
Age (years) | 63.4 ± 3.2 | 42.0 ± 2.9 | 43.3 ± 9.3 | <0.001a |
0.060b | ||||
0.86c | ||||
Gender (M/F) | 1/4 | 2/10 | 3/1 | |
FVC (% predicted) | 89.0 ± 10.5 | 67.8 ± 5.7 | 120.5 ± 3.6 | 0.073a |
0.037b | ||||
<0.001c | ||||
TLC (% predicted) | 87.0 ± 7.6 | 73.6 ± 6.1 | 115.3 ± 5.0 | 0.23a |
0.022b | ||||
0.002c | ||||
DLCOa (% predicted) | 55.4 ± 11.8 | 43.2 ± 4.6 | 92.5 ± 8.2 | 0.25a |
0.045b | ||||
<0.001c | ||||
HRCT scan | Fibrosis | Fibrosis | No fibrosis |
FPF, familial pulmonary fibrosis; HPSPF, Hermansky–Pudlak syndrome pulmonary fibrosis; URel, unaffected relative; M, male; F, female; FVC, forced vital capacity; TLC, total lung capacity; DLCOa, diffusion capacity (adjusted); HRCT, high-resolution computed tomography.
aFPF vs HPSPF.
bFPF vs URel.
cHPSPF vs URel.