Decreased Risk of Ischemic Heart Disease in Individuals with Severe Alpha 1-Antitrypsin Deficiency (PiZZ) in Comparison with the General Population

Figures & data

Table 1 Clinical Characteristics of the Population at Risk for Individuals with Severe Alpha-1-Antitrypsin Deficiency and for Population-Based Controls at Inclusion

	PiZZ N=1545	Controls N=5883	P-value
Men, n (%)	768 (50)	2977 (50)	0.540
Age in years, mean (± SD)	47 (± 17)	45 (±14)	<0.001
Smoking habits
Current smokers, n (%)	130 (8)	1528 (26)	<0.001
Ex-smokers, n (%)	702 (46)	1240 (21)
Never-smokers, n (%)	713 (46)	3115 (53)
Follow-up time in years, median (range)	11 (0.2–23)	18 (0.2–23)	<0.001
Deaths, n (%)	510 (33)	733 (12)	<0.001

Abbreviation: PiZZ, individuals with severe alpha 1-antitrypsin deficiency.

Table 2 Prevalence of Comorbidities at Inclusion for the PiZZ Individuals and the Controls; in the Total Population and Stratified by Smoking Habits

	All			Ever-Smokers			Never-Smokers
	PiZZ N=1545	Controls N=5883	P value	PiZZ n=832	Controls n=2768	P value	PiZZ n=713	Controls n=3115	P value
Hypertension	63 (4)	184 (3)	0.067	33 (4)	100 (4)	0.67	30 (4)	84 (3)	0.052
Diabetes mellitus	30 (2)	95 (2)	0.370	19 (2)	55 (2)	0.58	11(2)	40 (1)	0.59
Hyperlipidemia	24 (2)	52 (1)	0.070	14 (2)	23 (1)	0.05	10 (1)	29 (0.9 %)	0.30
COPD	711 (46)	219 (4)	<0.001	539 (65)	148 (5)	<0.001	172 (24)	71 (2)	<0.001

Note: Diagnoses were based on ICD 9 and 10 codes. Data presented as frequency (percentage).

Abbreviation: PiZZ, individuals with severe alpha 1-antitrypsin deficiency.

Figure 1 Cumulative incidence of ischemic heart disease in individuals with severe alpha-1-antitrypsin deficiency (PiZZ) and controls from the general population.

Table 3 Multivariate Analysis of Risk Factors for Incident Ischemic Heart Disease in the Total Study Population and in Stratified Analyses in AAT-Deficient Individuals and Controls from the General Population

	All		PiZZ Individuals		Controls
Variables	Hazard Ratio (95% CI)	P-value	Hazard Ratio (95% CI)	P-value	Hazard Ratio (95% CI)	P-value
Controls vs AATD	1.8 (1.4–2.3)	<0.001			–
Male vs Female	1.8 (1.6–2.1)	<0.001	1.4 (0.9–2.0)	0.078	1.9 (1.6–2.2)	<0.001
Age (per 1 year)	1.1 (1.1–1.1)	<0.001	1.1 (1.1–1.1)	<0.001	1.1 (1.1–1.1)	<0.001
Ever- vs non-smokers	1.3 (1.1–1.5)	<0.001	0.9 (0.7–1.5)	0.945	1.3 (1.1–1.5)	<0.001
COPD, yes vs no	1.7 (1.4–2.2)	<0.001	2.1 (1.3–3.2)	<0.010	1.8 (1.4–2.4)	<0.001
Hypertension, yes vs no	2.9 (2.2–3.7)	<0.001	1.5 (0.7–3.1)	0.249	3.3 (2.5–4.4)	<0.001
Diabetes, yes vs no	1.9 (1.4–2.6)	<0.001	2.9 (1.3–6.3)	<0.010	1.7(1.2–2.5)	<0.010
Hyperlipidemia, yes vs no	3.9 (2.5–6.1)	<0.001	6.6 (3.1–14.4)	<0.001	3.3 (1.9–5.6)	<0.001

Abbreviations: PiZZ, severe alpha 1-antitrypsin deficiency; HR, hazard ratio; CI, confidence interval.

Table 4 Multivariate Analysis of Risk Factors for Incident Ischemic Heart Disease (HR; 95% CI) in the Study Population as a Whole, Stratified by Smoking Habits

	Ever-Smokers N=3600		Never-Smokers N=3828
Variables	Hazard Ratio (95% CI)	P-value	Hazard Ratio (95% CI)	P-value
Controls vs PiZZ	2.1 (1.5–2.9)	<0.001	1.5 (1.1–2.2)	<0.050
Male vs Female	1.7 (1.4–2.1)	<0.001	1.9 (1.5–2.4)	<0.001
Age (per 1 year)	1.08 (1.07–1.09)	<0.001	1.1 (1.1–1.1)	<0.001
COPD, yes vs no	1.8 (1.4–2.5)	<0.001	1.6 (1.1–2.3)	<0.050
Hypertension, yes vs no	3.1 (2.1–4.2)	<0.001	2.8 (1.9–4.2)	<0.000
Diabetes, yes vs no	1.7 (1.1–2.5)	<0.010	2.6 (1.5–4.4)	<0.000
Hyperlipidemia, yes vs no	5.4 (3.1–9.4)	<0.001	2.6 (1.3–5.3)	<0.010

Abbreviations: PiZZ, severe alpha 1-antitrypsin deficiency; HR, hazard ratio; CI, confidence interval.