TABLE 4.
Forced vital capacity and diffusion capacity change over time for interstitial lung disease subjects with heterozygous TERT, TERC, RTEL1 or PARN mutations
| PFT parameter | Group | N | Mean number of measurements | Mean follow-up time years | Mean change per year | p-value |
|---|---|---|---|---|---|---|
| FVC L | All | 71 | 3.89 | 2.42 | −0.30 | |
| TERT | 46 | 3.76 | 2.16 | −0.30 | NS | |
| TERC | 6 | 4.67 | 2.23 | −0.26 | ||
| RTEL1 | 10 | 3.70 | 2.94 | −0.35 | ||
| PARN | 9 | 4.22 | 3.30 | −0.27 | ||
| FVC % predicted | All | 71 | 3.83 | 2.40 | −7.40 | |
| TERT | 46 | 3.67 | 2.13 | −7.48 | NS | |
| TERC | 6 | 4.67 | 2.23 | −6.65 | ||
| RTEL1 | 10 | 3.70 | 2.94 | −7.59 | ||
| PARN | 9 | 4.22 | 3.30 | −7.87 | ||
| DLCO mL·min−1·mmHg−1 | All | 67 | 3.39 | 2.30 | −1.73 | |
| TERT | 44 | 3.25 | 2.04 | −1.73 | NS | |
| TERC | 5 | 4.00 | 2.26 | −0.81 | ||
| RTEL1 | 9 | 3.11 | 2.62 | −2.11 | ||
| PARN | 9 | 4.00 | 3.30 | −1.80 | ||
| DLCO % predicted | All | 69 | 3.33 | 2.23 | −6.26 | |
| TERT | 46 | 3.15 | 1.94 | −6.35 | NS | |
| TERC | 5 | 4.00 | 2.26 | −2.53 | ||
| RTEL1 | 9 | 3.11 | 2.62 | −5.71 | ||
| PARN | 9 | 4.11 | 3.30 | −7.53 |
NS: nonsignificant; PFT: pulmonary function test; FVC: forced vital capacity; DLCO: diffusion capacity of the lung for carbon monoxide.