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Table 1 Participant demographics

From: Cardiovascular disease in Alpha 1 antitrypsin deficiency: an observational study assessing the role of neutrophil proteinase activity and the suitability of validated screening tools

 

AATD

Non-AATD COPD

Healthy controls

P value

Number

228

50

51

 

Sex (n male: female)

144:84

33: 17

28:23

0.4013

Age (years), median (IQR)

58 (48–65)

71 (67–77)

68 (60–77)

 < 0.0001*

PiZ

221

0

0

 

PiZ null

7

N/A

N/A

 

Blood AAT concentration

Median (IQR)

4.1μM (3.0–6.2)

26.1 mM

(21.3–29.0)

22.3 mM

(19.8–26.3)

 

Never smoked, n (%)

58 (25.4%)

0

9 (17.6%)

0.0003**

Ex-smoker, n (%)

165 (72.4%)

40 (80%)

38 (74.5%)

0.213**

Current smoker, n

5 (2.2%)

10 (20%)

4 (7.8%)

0.008**

Pack year history. Median (IQR)

12 (5–21)

44 (34–59)

21 (12–35)

 < 0.0001*

FEV1 (L) median (IQR)

1.6 (1.1–2.3)

1.3 (0.9–1.8)

2.8 (2.3–3.4)

 < 0.0001*

FEV1 (percent predicted). Median (IQR)

51.7 (36.2–81.3)

49.5 (40.0–72.0)

101 (92.0–112.5)

 < 0.0001*

FEV1/ FVC ratio. Median, (IQR)

46.4 (36.8–67.4)

52.0 (41.5–61.6)

78.9 (72.7–81.9)

 < 0.0001*

GOLD classification, n (%)

    

 Normal spirometry

 GOLD Mild

 GOLD Moderate

 GOLD Severe

 GOLD Very severe

54 (23.7%)

13 (5.7%)

56 (24.7%)

74 (32.5%)

31 (13.6%)

0

6 (12%)

19 (38%)

21 (42.0%)

4 (8.0%)

51 (100%)

0.2922**

Kco, % predicted. Median (IQR)

59.9 (48.3–74.9)

62 (47–77)

100.5 (82.5–109.75)

 < 0.0001*

Residual volume, % predicted. Median, (IQR)

108.7

(88.8–140.3)

117.5

(100.5–145)

77.0

(72.0–88.5)

 < 0.0001*

Reported exacerbation frequency in past 12 months

    

 Median (IQR)

 Frequent (2 or more), n (%)

 Infrequent (1), n (%)

 None, n (%)

1 (0–2)

82 (36.0%)

52 (22.8%)

94 (41.2%)

1.5 (1–3)

26 (52%)

14 (28%)

10 (20%)

N/A

0.0057*

Presence of Chronic Bronchitis

41(18.0%)

26 (52%)

0

 < 0.0001**

Presence of emphysema on CT

CT scans available (n)

Emphysema present (n (%)

167

94 (56.3%)

50

23 (46.0%)

N/A

0.195**

COPD Assessment Test. Median (IQR)

17 (12–23)

25 (21–28)

n/a

 < 0.0001*

Inhaled corticosteroid, n (%)

109 (48%)

25 (50%)

0

0.876**

Long-acting inhaled beta 2 agonist

148 (65%)

30 (59%)

0

0.249**

Long-acting inhaled anti-muscarinic

162 (71%)

37 (74%)

0

0.132**

BMI. Median (IQR) kg/m2

25 (23–28)

24 (23–26)

25 (23–29)

0.879*

Heart rate, bpm. Median (IQR)

65 (58–72)

71 (62.3–79.0)

64 (57–72)

0.003*

Systolic blood pressure, mmHg. Median (IQR)

135 (126–151)

142 (131–155)

130 (124–138)

0.0174*

Diastolic blood pressure, mmHg. Median (IQR)

81 (74–88)

79 (72–86)

78 (70–84)

0.08*

aPWV, m/s Median (IQR)

9.4 (8.3–11.0)

10.0 (9.4–12.0)

8.3 (7.2–9.4)

 < 0.0001*

QRISK2® Score. Median (IQR)

n = (excluding those with CVD)

9.5 (4.1–15.1)

n = 211

25 (17–32)

n = 41

12.7 (9.8–25.4)

n = 47

 < 0.0001*

  1. Patient demographics as collected on the day of cardiovascular risk assessment. PiZZ, and PiZ “Null” are the deficiency alleles present in this patient population. Lung function measures are post bronchodilation and include standard measures of spirometry (FEV1/FVC) as well as measures of transfer coefficient (Kco). AATD and COPD patients were stratified in accordance with GOLD groups
  2. BMI = Body Mass Index. QRISK2® Score was only calculated in those without a known history of CVD or a CVA as confirmed by self-report and General Practitioner records
  3. *Kruskal Wallis
  4. **Contingency (Fishers Exact or Chi squared). Bold results denote significant differences. Where significant differences were found across the three groups, Dunn’s multiple comparisons tests were performed between individual groups to determine which of these were significantly different. The significance values of these are described within the main body of the results
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172