Alpha-1 Antitrypsin Deficiency and Pulmonary Morbidity in Patients with Primary Immunodeficiency Disease: A Single-Center Experience

Table 2

Baseline characteristics of 110 patients with measured AAT levels.

	PID patients without AATD (n = 103)	PID patients with AATD (n = 7)
		ZZ (n = 2)	MZ (n = 5)
Clinical parameters
Median age (± SD)	49 (±16)	39 (±10)	43 (±13)
Male gender, N (%)	40 (39)	2 (100)	2 (40)
Female gender, N (%)	63 (61)	0	3 (60)
Alpha 1-antitrypsin level (mg/dl)
Median	147.5	39	86
Range	97−313	19−59	74−90
Respiratory parameters
Obstruction, N (%)	28 (27)	2 (100)	2 (40)
Restriction, N (%)	9 (9)	0	0
Radiologic parameters
Bronchiectasis, N (%)	18 (17)	0	0
Emphysema, N (%)		1 (50)	1 (20)
PID
CVID	41 (40)	1 (50)	1 (20)
IgA with IgG subclass deficiency	5 (5)	0	0
Unclassified antibody deficiency	30 (29)	1 (50)	1 (20)
Isolated IgG subclass deficiency	16 (16)	0	1 (20)
Hyper-IgM Syndrome	1 (1)	0	0
Hyper-IgE Syndrome	1 (1)	0	0
Selective IgM deficiency	6 (6)	0	2 (40)
Selective IgA deficiency	3 (3)	0	0