Characteristic, n (%) | All patients | European study | North American study | ||||||
---|---|---|---|---|---|---|---|---|---|
All (N = 113) | Treatment before study | All (n = 45) | Treatment before study | All (n = 68) | Treatment before study | ||||
IVIG (n = 76) | SCIG (n = 37) | IVIG (n = 30) | SCIG (n = 15) | IVIG (n = 46) | SCIG (n = 22) | ||||
Age, y | |||||||||
 0–12 | 29 (25.7) | 20 (26.3) | 9 (24.3) | 14 (31.1) | 12 (40.0) | 2 (13.3) | 15 (22.1) | 8 (17.4) | 7 (31.8) |
 13–17 | 18 (15.9) | 14 (18.4) | 4 (10.8) | 9 (20.0) | 7 (23.3) | 2 (13.3) | 9 (13.2) | 7 (15.2) | 2 (9.1) |
  ≥ 18 | 66 (58.4) | 42 (55.3) | 24 (64.9) | 22 (48.9) | 11 (36.7) | 11 (73.3) | 44 (64.7) | 31 (67.4) | 13 (59.1) |
Sex | |||||||||
 Female | 47 (41.6) | 31 (40.8) | 16 (43.2) | 16 (35.6) | 10 (33.3) | 6 (40.0) | 31 (45.6) | 21 (45.7) | 10 (45.5) |
 Male | 66 (58.4) | 45 (59.2) | 21 (56.8) | 29 (64.4) | 20 (67.7) | 9 (60.0) | 37 (54.4) | 25 (54.3) | 12 (54.5) |
Race | |||||||||
 Asian | 2(1.8) | 1 (1.3) | 1 (2.7) | 1 (2.2) | 0 (0.0) | 1 (6.7) | 1 (1.5) | 1 (2.2) | 0 (0.0) |
 Black | 3 (2.7) | 2 (2.6) | 1 (2.7) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 3 (4.4) | 2 (4.3) | 1 (4.5) |
 Other | 2 (1.8) | 0 (0.0) | 2 (5.4) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 2 (2.9) | 0 (0.0) | 2 (9.1) |
 White | 106 (93.8) | 73 (96.1) | 33 (89.2) | 44 (97.8) | 30 (100.0) | 14 (93.3) | 62 (91.2) | 43 (93.5) | 19 (86.4) |
Ethnicity | |||||||||
 Hispanic or Latino | 5 (4.4) | 5 (6.6) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 5 (7.4) | 5 (10.9) | 0 (0.0) |
 Non-Latino/Hispanic | 108 (95.6) | 71 (93.4) | 37 (100.0) | 45 (100.0) | 30 (100.0) | 15 (100.0) | 63 (92.6) | 41 (89.1) | 22 (100.0) |
Country | |||||||||
 Austria | 1 (0.9) | 0 (0.0) | 1 (2.7) | 1 (2.2) | 0 (0.0) | 1 (6.7) | – | – | – |
 Belgium | 1 (0.9) | 1 (1.3) | 0 (0.0) | 1 (2.2) | 1 (3.3) | 0 (0.0) | – | – | – |
 Canada | 2 (1.8) | 2 (2.6) | 0 (0.0) | – | – | – | 2 (2.9) | 2 (4.3) | 0 (0.0) |
 Germany | 12 (10.6) | 6 (7.9) | 6 (16.2) | 12 (26.7) | 6 (20.0) | 6 (40.0) | – | – | – |
 Hungary | 20 (17.7) | 19 (25.0) | 1 (2.7) | 20 (44.4) | 19 (63.3) | 1 (6.7) | – | – | – |
 Netherlands | 2 (1.8) | 1 (1.3) | 1 (2.7) | 2 (4.4) | 1 (3.3) | 1 (6.7) | – | – | – |
 Sweden | 3 (2.7) | 1 (1.3) | 2 (5.4) | 3 (6.7) | 1 (3.3) | 2 (13.3) | – | – | – |
 United Kingdom | 6 (5.3) | 2 (2.6) | 4 (10.8) | 6 (13.3) | 2 (6.7) | 4 (26.7) | – | – | – |
 United States | 66 (58.4) | 44 (57.9) | 22 (59.5) | – | – | – | 66 (97.1) | 44 (95.7) | 22 (100.0) |
Site of care | |||||||||
 Home | 46 (40.7) | 12 (15.8) | 34 (91.9) | 18 (40.0) | 3 (10.0) | 15 (100.0) | 28 (41.2) | 9 (19.6) | 19 (86.4) |
 Other | 67 (59.3) | 64 (84.2) | 3 (8.1) | 27 (60.0) | 27 (90.0) | 0 (0.0) | 40 (58.8) | 37 (80.4) | 3 (13.6) |
PID diagnosis | |||||||||
 Common variable immune deficiency (including familial TACI mutation C.512 > G) | 48 (42.5) | 33 (43.4) | 15 (40.5) | 28 (62.2) | 18 (60.0) | 10 (66.7) | 20 (29.4) | 15 (32.6) | 5 (22.7) |
 Congenital agammaglobulinemia - X-linked or autosomal recessive | 21 (18.6) | 13 (17.1) | 8 (21.6) | 10 (22.2) | 6 (20.0) | 4 (26.7) | 11 (16.2) | 7 (15.2) | 4 (18.2) |
 Hyper-IgM – X-linked or autosomal recessive | 5 (4.4) | 5 (6.6) | 0 (0.0) | 3 (6.7) | 3 (10.0) | 0 (0.0) | 2 (2.9) | 2 (4.3) | 0 (0.0) |
 IgG subclass deficiency – isolated, or with low IgG | 1 (0.9) | 0 (0.0) | 1 (2.7) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (1.5) | 0 (0.0) | 1 (4.5) |
 IgM and IgG deficiencies | 1 (0.9) | 0 (0.0) | 1 (2.7) | 1 (2.2) | 0 (0.0) | 1 (6.7) | 0 (0.0) | 0 (0.0) | 0 (0.0) |
 Severe combined immunodeficiency | 1 (0.9) | 0 (0.0) | 1 (2.7) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (1.5) | 0 (0.0) | 1 (4.5) |
 Specific antibody deficiency – isolated, or with hypogammaglobulinemia or IgG subclass deficiency | 36 (31.9) | 25 (32.9) | 11 (29.7) | 3 (6.7) | 3 (10.0) | 0 (0.0) | 33 (48.5) | 22 (47.8) | 11 (50.0) |