Alpha-1 Antitrypsin Augmentation Therapy Improves Survival in Severely Deficient Patients with Predicted FEV1 Between 10% and 60%: A Retrospective Analysis of the NHLBI Alpha-1 Antitrypsin Deficiency Registry

Figures & data

Figure 1 Study enrolment and changes form original analysis.

Table 1 Characteristics of the Study Cohort by Decile of FEV1 and Morbidity Status*

FEV1	Status	Number N=1126	Age (Years)	Male Sex (%)	DLCO % Predicted N=885	TLC % Predicted N=850	BMI^†
<10%	Alive	1	45.1	1 (100.0)	4.4	NA	27.9
	Deceased	5	46.3 ± 5.1	3 (60.0)	9.0 ± 2.9	89.5 ± 23.4	18.2 ± 3.0
10–20%	Alive	90	46.0 ± 7.3	55 (61.1)	28.8 ± 12.8	113.1 ± 23.7	24.4 ± 4.9
	Deceased	65	48.6 ± 9.2	41 (63.0)	22.1 ± 11.6	109.8 ± 29.3	22.8 ± 4.6
20–30%	Alive	194	46.1 ± 8.3	113 (58.3)	38.1 ± 14.3	110.5 ± 25.6	25.7 ± 5.5
	Deceased	70	51.6 ±10.7	37 (52.9)	33.7 ± 15.5	104.7 ± 28.8	23.9 ± 5.3
30–40%	Alive	195	46.8 ± 9.1	127 (65.1)	46.7 ± 14.0	110.3 ± 20.3	26.1 ± 5.3
	Deceased	34	56.2 ± 11.0	21 (61.8)	36.4 ± 14.0	107.1 ± 33.1	25.6 ± 5.0
40–50%	Alive	99	48.7 ± 10.6	53 (53.5)	49.9 ± 14.5	108.6 ± 24.4	26.0 ± 4.7
	Deceased	13	54.9 ± 10.9	6 (46.2)	52.6 ± 13.2	112.0 ± 17.6	26.1 ± 5.4
50–60%	Alive	73	48.3 ± 10.7	41 (56.2)	56.2 ± 15.1	110.6 ± 23.4	26.5 ± 4.5
	Deceased	7	59.6 ± 14.5	4 (57.1)	58.4 ± 9.4	107.6 ± 19.0	25.6 ± 5.6
>60%	Alive	268	41.7 ± 11.4	112 (41.8)	74.3 ± 18.9	107.0 ± 17.6	27.2 ± 5.4
	Deceased	12	50.5 ± 11.1	12 (100.0)	73.4 ± 15.6	109.4 ± 12.5	30.0 ± 7.1

Notes: *Plus-minus values are means ±SD. ^†Data is reported for 1126 individuals for age, sex, and BMI.

Abbreviation: NA, not available.

Figure 2 Kaplan–Meier survival curves for all study individuals with baseline FEV1 data (N=1126).

Notes: The cohort treated with sometimes or always augmentation therapy (―) in red had improved survival compared to those without augmentation therapy (―) in green stratified by FEV1% predicted deciles between 10% and 60%. Graphs censored at 2500 days.