Long-term evolution of lung function in individuals with alpha-1 antitrypsin deficiency from the Spanish registry (REDAAT)

Figures & data

Table 1 Baseline characteristics of PI*ZZ individuals registered in REDAAT (N=450)

Variable	Individuals with lung function follow-up, n=122	Individuals without lung function follow-up, n=328	p-values
Age (years)	61.6 (10)	52.2 (16.2)	0.001
Sex (male)	68 (58)	124 (38.3)	0.241
BMI (kg/m^2)	25.3 (4.3)	25.1 (4.6)	0.515
Tobacco consumption
Never smokers	21 (16.2)	65 (19.8)	0.562
Current smokers	8 (6.2)	28 (8.7)
Former smokers	101 (77.7)	234 (71.5)
Pack/year	25.8 (17.4)	27 (20)
Age at diagnosis (years)	54.2 (11.2)	50 (11.7)	0.002
Diagnosis cause
Pulmonary disease	95 (73.1)	196 (60)	0.005
Family screening	27 (20.8)	62 (18.8)
Age at onset of symptoms	38.6 (12.2)	35.3 (16.2)	0.012
Clinical presentation
Emphysema	108 (83.1)	198 (60.4)	<0.001
Chronic bronchitis	58 (44.6)	111 (34.2)	0.044
Bronchiectasis	55 (42.3)	71 (21.6)	<0.001
Asthma	26 (20)	62 (19.8)	0.976
Other	16 (12.3)	36 (11.3)	0.960
Previous pneumonia	40 (30.8)	72 (22.1)	0.064
Baseline FVC (L)	3.23 (1.2)	3.55 (1.2)	0.044
Baseline FVC (%)	70.5 (22)	71.8 (25)	0.035
Baseline FEV1 (L)	1.87 (0.9)	2.26 (1.2)	0.001
Baseline FEV1 (%)	60.8 (30.6)	67.5 (35.3)	0.001
Respiratory treatment	109 (83.8)	206 (63)	<0.001
LTOT	21 (16.2)	36 (11.8)	0.278
Augmentation therapy	77 (59.2)	82 (25.3)	<0.001
Length of follow-up (years)	12.3 (4.2)	3.5 (3.7)	<0.001

Notes: Data are expressed as mean (SD) or n (%). Values in bold indicate statistically significant.

Abbreviations: FEV₁, forced expiratory volume in the first second; FVC, forced vital capacity; LTOT, long-term oxygen therapy; REDAAT, Alpha-1 Antitrypsin Deficiency Spanish Registry.

Figure 1 Mean FEV₁ decline according to quartiles of decline (n=122).

Abbreviations: FEV₁, forced expiratory volume in the first second; Q, quartile.

Table 2 Baseline characteristics according to quartiles of FEV₁ decline (n=122)

Variable	Q1, n=30	Q2, n=30	Q3, n=31	Q4, n=31	p-value
Age at diagnosis (years)	47.6 (14.5)	45.4 (10)	46.8 (12)	45.3 (9.8)	0.849
Sex (male)	20 (68.7)	14 (46.7)	13 (40.6)	21 (70)	0.051
BMI (kg/cm^2)	24.5 (4.1)	26.2 (5.4)	25.7 (4.5)	25.4 (3.7)	0.762
Tobacco consumption
Never smokers	5 (16.7)	4 (13.3)	5 (15.6)	7 (23.3)	0.325
Current smokers	0	1 (3.3)	3 (8.4)	4 (13.3)	0.961
Former smokers	25 (83.3)	25 (83.3)	24 (75)	19 (63.3)
Pack/year	27.3 (18.7)	23.5 (14.2)	26.3 (19.4)	24.2 (14.4)
Clinical presentation
Emphysema	25 (83.3)	25 (83.3)	4 (75)	26 (86.7)	0.665
Chronic bronchitis	18 (60)	10 (33.3)	11 (34.4)	15 (50)	0.105
Bronchiectasis	17 (56.7)	14 (46.7)	10 (31.3)	13 (43.3)	0.246
Asthma	5 (16.7)	7 (23.3)	6 (18.8)	6 (20)	0.931
Previous pneumonia	9 (30)	7 (23.3)	12 (37.5)	9 (30)	0.688
Baseline FVC (L)	3.034 (1.0)	2.941 (0.9)	3.223 (1.1)	3.904 (1.1)	0.025
Baseline FVC (%)	67.4 (18.7)	69.2 (22.0)	71.2 (20.1)	72.2 (18.2)	0.044
Baseline FEV1 (L)	1.645 (1.1)	1.610 (0.7)	1.998 (0.9)	2.331 (0.8)	0.002*
Baseline FEV1 (%)	52 (28.3)	56.2 (25.1)	60.2 (29.3)	62.6 (32.1)	0.023*
Respiratory treatment	25 (83.3)	27 (90)	24 (75)	27 (90)	0.857
LTOT	2 (6.7)	6 (20)	6 (18.8)	5 (16.7)	0.470
Augmentation therapy	18 (60)	21 (70)	13 (40)	19 (63.3)	0.205
Length of follow-up (years)	10.4 (5)	11 (6.2)	12.8 (7.8)	14.3 (5.1)	0.089
Δ FEV1 year (mL/year)	35.1 (20.7)	−21.1 (8.22)	−42.8 (7.1)	−83.5 (32.3)	,0.001

Notes:

* Linear trend <0.05. Values in bold indicate statistically significant.

Abbreviations: BMI, body mass index; FEV₁, forced expiratory volume in the first second; FVC, forced vital capacity; LTOT, long-term oxygen therapy; Q, quartile.

Table 3 Unadjusted and adjusted linear regression model for ΔFEV₁ (mL/year)

Variable	Unadjusted beta coefficient	p-values	95% CI	Adjusted beta coefficient	p-values	95% CI
Age at inclusion (years)	0.150	0.724	−1.005 to 0.701
Age of diagnosis (years)	0.170	0.665	0.443–0.667
Sex (male)	2.088	0.835	−17.7 to 21.90
BMI (kg/m^2)	0.369	0.324	−1.9 to 2.61
Current and former smoker	22.5	0.038	1.320–43.7	19.8	0.001	10.7–29.5
Asthma	−10.7	0.389	−35.4 to 13.9
Emphysema	−2.9	0.825	−28.4 to 22.7
Chronic bronchitis	−2.11	0.833	−21.9 to 17.7
Bronchiectasis	−13.3	0.181	−32.9 to 6.3
Previous pneumonia	9.462	0.099	−11.852 to 30.8	27.90	0.026	3.44–52.3
Baseline FEV1 (%)	−1.92	0.092	−0.5 to 0.129	0.798	0.010	0.157–0.939
Respiratory treatment	20.4	0.134	−6.4 to 47.3
LTOT	17.8	0.193	−9.1 to 44.7
Augmentation therapy	−4.2	0.675	−24.2 to 15.7

Note: Values in bold indicate statistically significant.

Abbreviations: BMI, body mass index; FEV₁, forced expiratory volume in the first second; LTOT, long-term oxygen therapy.