Table 3 Lung disease characteristics of PI*Mmalton (moderate and severe) and PI*ZZ patients

Reasons that led to the diagnosis

 Respiratory symptoms

16

(61.5%)

24

(72.7%)

540

(65.3%)

0.620

 Liver symptoms

3

(11.5%)

1

(3.0%)

68

(8.2%)

n.a

 Family screening

4

(15.4%)

5

(15.2%)

138

(16.7%)

0.960

 Other

3

(11.5%)

4

(12.1%)

54

(6.5%)

n.a

Symptoms at presentation

 Dyspnea, n (%)

14

(53.8%)

21

(63.6%)

527

(63.7%)

0.588

 Cough, n (%)

9

(34.6%)

8

(24.2%)

269

(32.5%)

0.588

 Sputum, n (%)

3

(11.5%)

5

(15.2%)

184

(22.2%)

0.278

 Jaundice, n (%)

1

(3.8%)

0

(0.0%)

15

(1.8%)

n.a

 Abnormal liver function test, n(%)

3

(11.5%)

1

(3.0%)

35

(4.2%)

n.a

 Other, n (%)

1

(3.8%)

2

(6.1%)

55

(6.7%)

n.a

 Asymptomatic, n (%)

7

(26.9%)

8

(24.2%)

122

(14.8%)

0.087

 Lung disease, n (%)

13

(50.0%) ^a

27

(81.8%)^b

646

(83.6%) ^b

 < 0.001²

 COPD³, n (%)

8

(30.8%)

20

(60.6)%

429

(51.9%)

0.060

 Emphysema³, n (%)

9

(34.6%) ^a

18

(54.5%)^a,b

506

(61.2%) ^b

0.019²

 Chronic Bronchitis³, n (%)

2

(7.7%)

1

(3.0%)

50

(6.0%)

n.a

 Bronchiectasis³, n (%)

2

(7.7%)

6

(18.2%)

183

(22.1%)

0.188

 Asthma³, n (%)

2

(7.7%)

2

(6.1%)

120

(14.5%)

n.a

 Lung cancer³, n(%)

0

(0.0%)

0

(0.0%)

1

(0.1%)

n.a

 Lung disease type (Other)³, n(%)

1

(3.8%)

0

(0.0%)

22

(2.7%)

n.a

Exacerbations/Respiratory Infections

 Ambulatory exacerbations (Last 12 months) ≥ 2, n(%)

3

(11.5%)

1

(3.1%)

95

(11.6%)

n.a

 Admissions in hospital related to COPD (Last 12 months) ≥ 1, n(%)

0

(0.0%)

2

(6.5%)

66

(8.1%)

n.a

 Had pneumonia, n(%)

6

(24.0%)

11

(34.4%)

226

(31.6%)

0.679

 No. episodes, Median (IQR)

1

(1–3)

1

(1–1)

1

(1–2)

0.628

Functional Respiratory Tests

 FEV1 (%) (Prebronchodilator) Mean ± SD

85.3

 ± 28.5

62.2

 ± 24.6

71.5

 ± 30.5

0.058

 FVC (%) (Prebronchodilator), Mean ± SD

100.5

 ± 21.6

83.1

 ± 23.5

94.5

 ± 23.3

0.051

 FEV1/FVC (%) (Prebronchodilator), Mean ± SD

69.0^a

 ± 18.0

55.9^b

 ± 17.8

57.6^b

 ± 19.4

0.018²

 DLCO (%), Mean ± SD

77.0^a

 ± 30.9

67.2^a,b

 ± 20.9

63.3^b

 ± 24.9

0.031²

 KCO (%), Mean ± SD

77.9

 ± 34.4

68.9

 ± 26.2

67.7

 ± 22.9

0.140

Comorbidities

 Charlson Comorbidity Index, Mean ± SD

3.4

 ± 2.7

3.0

 ± 1.8

3.2

 ± 2.0

0.792

Symptom Scores

 BODEX, Mean ± SD

1.0

 ± 1.3

2.0

 ± 1.6

2.0

 ± 2.0

0.054

 CAT, Mean ± SD

7.2^a

 ± 6.8

9.4^a,b

 ± 8.1

13.5^b

 ± 9.6

 < 0.001²

 If dyspnea(current symptoms): mMRC, Median (IQR)

2

1–2

2

1–2

2

1–2

0.355