Table 1.
Baseline characteristics of patients
| Overall n = 222 |
Familial n = 104 |
Sporadic n = 118 |
P value | |
|---|---|---|---|---|
| Age at diagnosis (years) | 66.5 ± 10.0 | 63.2 ± 11.2 | 69.4 ± 7.9 | < 0.001u |
| Gender (Female) | 62 (28) | 36 (35) | 26 (22) | 0.037χ |
| Comorbidities (Yes) | 164 (74) | 60 (58) | 104 (88) | < 0.001χ |
| Smoking history (Yes) | 130 (59) | 47 (45) | 83 (70) | < 0.001χ |
| Antifibrotic drugs (Yes) | 130 (59) | 60 (58) | 70 (59) | 0.806χ |
| Prednisone (Yes) | 80 (36) | 43 (41) | 37 (31) | 0.122χ |
| Idiopathic pulmonary fibrosis | 122 (55) | 44 (42) | 78 (66) | < 0.001χ |
| Radiological Pattern | n = 221 | n = 104 | n = 117 | < 0.001χ |
| Non-UIP | 86 (39) | 52 (50) | 34 (29) | |
| Indeterminate UIP | 27 (12) | 21 (20) | 6 (5) | |
| Probable UIP | 63 (29) | 12 (12) | 51 (44) | |
| UIP | 45 (20) | 19 (18) | 26 (22) | |
| Relative Telomere Length | n = 149 | n = 102 | n = 47 | - |
| p75-p100 | 1 (1) | 1 (1) | 0 (0) | |
| p50-75 | 18 (12) | 13 (13) | 5 (11) | |
| p25-50 | 45 (30) | 18 (18) | 27 (57) | |
| <p25 | 43 (29) | 34 (33) | 9 (19) | |
| <p10 | 17 (11) | 15 (15) | 2 (4) | |
| <p1 | 25 (17) | 21 (20) | 4 (9) | |
| Telomere shortening < p25 (Yes) | 85 (57) | 70 (69) | 15 (32) | < 0.001χ |
| Pathology Pattern | n = 94 | n = 41 | n = 53 | - |
| UIP | 32 (34) | 18 (44) | 14 (26) | |
| Probable UIP | 12 (13) | 8 (20) | 4 (8) | |
| Non-UIP | 50 (53) | 15 (36) | 35 (66) | |
| Associated lung cancer (Yes) | n = 219 | n = 104 | n = 115 | 0.314f |
| 9 (4) | 6 (6) | 3 (3) | ||
| Other cancer (Yes) | n = 219 | n = 104 | n = 115 | 0.634χ |
| 23 (10) | 12 (12) | 11 (10) | ||
| Telomere-related extrapulmonary manifestations (Yes) | n = 221 | n = 104 | n = 117 | < 0.001χ |
| 59 (27) | 43 (41) | 16 (14) | ||
| Blood count abnormality at diagnosis (Yes) | n = 218 | n = 103 | n = 115 | < 0.001χ |
| 55 (25) | 43 (42) | 12 (10) | ||
| Anaemia (Yes) | n = 219 | n = 104 | n = 115 | 0.015χ |
| 24 (11) | 17 (16) | 7 (6) | ||
| Thrombocytopenia (Yes) | n = 220 | n = 104 | n = 118 | 0.021χ |
| 16 (7) | 12 (12) | 4 (3) | ||
| Liver abnormality at diagnosis (Yes) | n = 220 | n = 104 | n = 116 | < 0.001χ |
| 59 (27) | 43 (41) | 16 (14) | ||
| Pulmonary Function Tests | ||||
| FVC (L) at diagnosis | n = 215 | n = 102 | n = 113 | 0.633t |
| 2.82 ± 0.88 | 2.85 ± 0.98 | 2.79 ± 0.784 | ||
| FVC (%) at diagnosis | n = 214 | n = 102 | n = 112 | 0.147t |
| 84.2 ± 20.1 | 86.3 ± 21.6 | 82.3 ± 18.4 | ||
| DLCO (%) at diagnosis | n = 213 | n = 101 | n = 112 | 0.726t |
| 59.4 ± 22.2 | 60.0 ± 22.5 | 58.9 ± 22.1 | ||
| Multidisciplinary committee (Yes) | n = 222 | n = 104 | n = 118 | 0.025χ |
| 187 (83) | 94 (90) | 93 (79) | ||
| Telomere-related gene variants (Yes) | n = 182 | n = 103 | n = 79 | < 0.001χ |
| 83 (46) | 66 (64) | 17 (22) | ||
| Family History | - | |||
| 1st degree relative (Yes) | - | 98 (94) | - | |
| 2nd degree relative (Yes) | - | 18 (17) | - | |
| More than 1 relative (Yes) | - | 49 (47) | - |
The results are given with 3 decimals: mean ± standard deviation if numerical and n (%) if categorical. tt-test, uMann-Whitney U test, χchi-square test, fFisher’s exact test