Table I.

Baseline characteristics of the 631 participants eligible for primary end-point evaluation

Characteristic	Patients with IEI (N = 456)	Controls (N = 175)	P value
Sex: male, n (%)	184 (40.4)	100 (57.1)	.00015∗
Age (y), mean ± SD	49.0 ± 14.9	51.6 ± 13.8	.052†
IEI diagnosis, n (%)
Antibody deficiency
CVID	196 (43.0)
Isolated IgG subclass deficiency ± IgA deficiency	121 (26.5)
SPAD	58 (12.7)
Undefined antibody deficiency‡	16 (3.5)
Absent B cells§
XLA	19 (4.2)
Autosomal-dominant agammaglobulinemia	1 (0.2)
CID	22 (4.8)
Phagocyte defects	15 (3.3)
Other‖	8 (1.7)
Immunoglobulin replacement therapy, n (%)	319 (70.0)	0
Noninfectious complications, n (%)
Autoimmune cytopenias	26 (5.7)	0
Other autoimmune diseases	71 (15.6)	1 (0.6)
Enteropathies	42 (9.2)	2 (1.1)
Lymphoproliferative diseases	33 (7.2)	0
Granulomatous-lymphocytic interstitial lung disease	30 (6.6)	0
Granulomatous complications affecting other organs	11 (2.4)	0
Malignancies	21 (4.6)	2 (1.1)
Most frequent other comorbidities, n (%)
Asthma	8 (1.7)	3 (1.7)
Bronchiectasis	10 (2.2)	0
Cardiac diseases¶	5 (1.1)	5 (2.9)
Diabetes	2 (0.4)	2 (1.1)
Hypertension	6 (1.3)	17 (9.7)
Immunosuppressive medication in past 2 y, n (%)	96 (21.1)	5 (2.9)	<.0001∗

^∗Pearson’s χ² test.

^†Independent t test.

^‡Patients with primary hypogammaglobulinemia and intact cellular immunity who do not fulfill diagnostic criteria of any of the other primary antibody deficiencies.

^§In the text, this cohort is referred to as XLA, although it also includes 1 participant with autosomal-dominant agammaglobulinemia (TCF3 mutation).

^‖Patients with an unknown classification of their IEI, high B-cell numbers, or hyper-IgM syndrome.

^¶Including myocardial infarction, chemotherapy-induced cardiomyopathy, coronary artery bypass grafting, arrhythmias, and heart valve diseases.