Table 1.
Patient characteristics.
| All patients (n = 20) | With telomere shortening (n = 12) | Without telomere shortening (n = 8) | P-value | |
|---|---|---|---|---|
| Age at lung transplant [years, mean (SD)] | 56.6 (9.39) | 51.8 (9.18) | 63.8 (3.11) | 0.001 |
| Gender (n, %) | ||||
| - Males | 15 (75.0) | 9 (75.0) | 6 (75.0) | 1.000 |
| - Females | 5 (25.0) | 3 (25.0) | 2 (25.0) | |
| Smoking history at diagnosis (n, %): | ||||
| - Non-smoker | 3 (15.0) | 2 (16.7) | 1 (12.5) | 1.000 |
| - Current smoker | 3 (15.0) | 2 (16.7) | 1 (12.5) | |
| - Former smoker | 14 (70.0) | 8 (66.7) | 6 (75.0) | |
| Diagnosis (n, %): | ||||
| - IPF | 8 (40.0) | 4 (33.3) | 4 (50.0) | 0.961 |
| - FPF | 8 (40.0) | 5 (41.6) | 3 (37.5) | |
| - HP | 1 (5.0) | 0 (0.0) | 1 (12.5) | |
| - CPFE | 1 (5.0) | 1 (8.3) | 0 (0.0) | |
| - DIP | 1 (5.0) | 1 (8.3) | 0 (0.0) | |
| - CTD-ILD | 1 (5.0) | 1 (8.3) | 0 (0.0) | |
| Pre-transplant treatment (n, %): | ||||
| - Pirfenidone | 7 (35.0) | 2 (16.6) | 5 (62.5) | 0.062 |
| - Nintedanib | 5 (25.0) | 3 (25.0) | 2 (25.0) | 1.000 |
| - Corticosteroids | 4 (20.0) | 2 (16.6) | 2 (25.0) | 1.000 |
| - Corticosteroids + immunosuppressive drug | 5 (25.0) | 5 (41.6) | 0 (0.0) | 0.055 |
| Telomere shortening (n, %): | ||||
| - n, below 1st percentile | 3 (25.0) | 3 (25.0) | 0 (0.0) | NA |
| - n, below 10th percentile | 4 (33.3) | 4 (33.3) | 0 (0.0) | |
| - n, below 25th percentile | 5 (41.7) | 5 (41.7) | 0 (0.0) | |
| Relative telomere length [mean, (SD)] | 1.13 (0.33) | 0.95 (0.29) | 1.40 (0.17) | 0.001 |
| Z-score [mean, (SD)] | −1.15 (1.22) | −1.83 (1.04) | −0.006 (0.44) | 0.001 |
| Likely causal variants in telomere-maintenance genes (n =1 8) (n, %): | 11 (55.0) | 10 (83.3) | 1 (12.5) | NA |
| - RTEL1 | 5 (45.5) | 5 (50.0) | 0 (0.0) | |
| - DKC1 | 2 (18.2) | 2 (20.0) | 0 (0.0) | |
| - PARN | 1 (09.1) | 0 (0.0) | 1 (100) | |
| - TERT | 3 (27.3) | 3 (30.0) | 0 (0.0) |
IPF, idiopathic pulmonary fibrosis; FPF, familial pulmonary fibrosis; HP, hypersensivity pneumonitis; CPFE, combined pulmonary fibrosis and emphysema; DIP, desquamative interstitial pneumonia; CTD-ILD, connective tissue disease-associated interstitial lung disease; NA, not applicable.