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Table 3 Characteristics of PI*ZZ patients according to their impairment in FEV1(%), KCO(%) or both

From: Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Variables

Impaired PFTs (n = 248)

Impaired KCO(%) (n = 69)

Impaired FEV1(%) (n = 37)

Normal PFTs (n = 95)

p value

Age, years

58.7 (10.7)

55.2 (13.4)

56.2 (12.4)

45.2 (14.9)

< 0.001cef

Sex, male (%)

145 (58.5)

33 (47.8)

25 (67.6)

43 (45.3)

0.035

BMI (kg/m2)

26.1 (5.0)

25.8 (4.5)

28 (5.2)

26.1 (4.6)

0.105

Smokers (%)

4 (1.6)

2 (2.9)

1 (2.7)

2 (2.1)

0.903

Ex-smokers (%)

193 (77.8)

28 (40.6)

22 (59.5)

23 (24.2)

< 0.001acf

Never smokers (%)

51 (20.6)

39 (56.5)

14 (37.8)

70 (73.7)

< 0.001acf

Tobacco Exposure (Pack-years)

21 (14.8)

12.7 (10.8)

14 (10.2)

6.9 (7.6)

< 0.001ac

Working status

 Active

107 (43.1)

40 (58)

20 (54.1)

74 (77.9)

< 0.001cef

 Retired

121 (48.8)

23 (33.3)

16 (43.2)

12 (12.6)

< 0.001cef

 Unemployed

14 (5.6)

2 (2.9)

1 (2.7)

2 (2.1)

0.424

 Other

6 (2.4)

4 (5.9)

0 (0)

7 (7.4)

0.077

Age at diagnosis of AATD

48.4 (12.8)

45.7 (18.3)

47.1 (12.7)

31.4 (22.5)

< 0.001cef

Reasons for diagnosis

 

 Index case (%)

202 (81.5)

40 (58.0)

24 (64.9)

48 (51.1)

< 0.001ac

 Family screening (%)

24 (9.7)

19 (27.5)

6 (16.2)

30 (31.6)

< 0.001ac

Clinical characteristics

 

 Chronic respiratory disease (%)

247 (99.6)

48 (70.6)

32 (86.5)

43 (45.7)

< 0.001abcef

 COPD (%)

214 (83.3)

19 (27.9)

25 (67.6)

5 (5.3)

< 0.001abcdef

 Emphysema (%)

203 (81.9)

30 (44.1)

27 (73.0)

10 (10.6)

< 0.001acdef

 Bronchiectasis (%)

45 (18.1)

23 (33.8)

15 (40.5)

18 (19.1)

0.002ab

 Asthma (%)

27 (10.9)

8 (11.8)

9 (24.3)

19 (20.2)

0.036

 Liver disease (%)

35 (14.5)

18 (26.1)

5 (13.5)

19 (20.2)

0.113

 Charlson index (age corrected)

4 (1.9)

2.8 (1.9)

2.9 (1.3)

1.5 (1.4)

< 0.001acef

 Cardiovascular disease (%)

80 (32.7)

13 (18.8)

10 (27)

12 (12.6)

0.001c

 Exacerbations previous year (%)

103 (41.5)

13 (18.8)

12 (32.4)

8 (8.4)

< 0.001acf

 History of pneumonia (%)

72 (31.2)

18 (26.9)

10 (30.3)

18 (19.6)

0.209

  1. Impairment is considered when values are < 80% of reference
  2. Data are presented a n (% of patients with non-missing data) or mean (Standard deviation)
  3. LFT: Lung function tests; FEV1: Forced expiratory volume in 1 s; KCO: Carbon monoxide transfer coefficient; BMI: Body mass index; COPD: Chronic obstructive pulmonary disease; AATD: Alpha-1 antitrypsin deficiency; cardiovascular disease includes hypertension, ischaemic heart disease, congestive heart failure and peripheral vascular disease
  4. Bonferroni ap < 0.05 for comparison between the groups “Impaired PFTs and Impaired KCO(%)”. bp < 0.05 for comparison between the groups “Impaired PFTs and impaired FEV1(%)”. cp < 0.05 for comparison between the groups “Impaired PFTs and normal PFTs”. dp < 0.05 for comparison between the groups “Impaired KCO(%) and impaired FEV1(%)”. ep < 0.05 for comparison between the groups “Impaired KCO(%) and normal PFTs”. fp < 0.05 for comparison between the groups “Impaired FEV1(%) and normal PFTs”