Table 1 Baseline characteristics of patients

Age at diagnosis (years)

66.5 ± 10.0

63.2 ± 11.2

69.4 ± 7.9

< 0.001^u

Gender (Female)

62 (28)

36 (35)

26 (22)

0.037^χ

Comorbidities (Yes)

164 (74)

60 (58)

104 (88)

< 0.001^χ

Smoking history (Yes)

130 (59)

47 (45)

83 (70)

< 0.001^χ

Antifibrotic drugs (Yes)

130 (59)

60 (58)

70 (59)

0.806^χ

Prednisone (Yes)

80 (36)

43 (41)

37 (31)

0.122^χ

Idiopathic pulmonary fibrosis

122 (55)

44 (42)

78 (66)

< 0.001^χ

Radiological Pattern

n = 221

n = 104

n = 117

< 0.001^χ

 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)

< 0.001^χ

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

0.314^f

9 (4)

6 (6)

3 (3)

Other cancer (Yes)

n = 219

n = 104

n = 115

0.634^χ

23 (10)

12 (12)

11 (10)

Telomere-related extrapulmonary manifestations (Yes)

n = 221

n = 104

n = 117

< 0.001^χ

59 (27)

43 (41)

16 (14)

Blood count abnormality at diagnosis (Yes)

n = 218

n = 103

n = 115

< 0.001^χ

55 (25)

43 (42)

12 (10)

Anaemia (Yes)

n = 219

n = 104

n = 115

0.015^χ

24 (11)

17 (16)

7 (6)

Thrombocytopenia (Yes)

n = 220

n = 104

n = 118

0.021^χ

16 (7)

12 (12)

4 (3)

Liver abnormality at diagnosis (Yes)

n = 220

n = 104

n = 116

< 0.001^χ

59 (27)

43 (41)

16 (14)

Pulmonary Function Tests

 FVC (L) at diagnosis

n = 215

n = 102

n = 113

0.633^t

2.82 ± 0.88

2.85 ± 0.98

2.79 ± 0.784

 FVC (%) at diagnosis

n = 214

n = 102

n = 112

0.147^t

84.2 ± 20.1

86.3 ± 21.6

82.3 ± 18.4

 DLCO (%) at diagnosis

n = 213

n = 101

n = 112

0.726^t

59.4 ± 22.2

60.0 ± 22.5

58.9 ± 22.1

Multidisciplinary committee (Yes)

n = 222

n = 104

n = 118

0.025^χ

187 (83)

94 (90)

93 (79)

Telomere-related gene variants (Yes)

n = 182

n = 103

n = 79

< 0.001^χ

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)

-