Table 1.
All, N = 38 | Discontinued | ||
---|---|---|---|
No, N = 23 | Yes, N = 15 | ||
Age, years, mean (SD) | 45.0 (21.6) | 47.7 (23.8) | 41.3 (16.9) |
Min, max | 8, 78 | 8, 78 | 15, 62 |
Age by category, n (%) | |||
≥ 16 years | 33 (86.8) | 19 (82.6) | 14 (93.3) |
< 16 years | 5 (13.2) | 4 (17.4) | 1 (6.6) |
Sex, n (%) | |||
Female | 27 (71.1) | 17 (74.0) | 10 (66.6) |
Male | 11 (28.9) | 6 (26.0) | 5 (33.3) |
PID diagnosis, n (%) | |||
CVID (any) | 27 (71.1) | ||
CVID B cell predominant | 12 (31.6) | 8 (34.8) | 4 (26.7) |
CVID not otherwise specified | 12 (31.6) | 5 (21.7) | 7 (46.7) |
CVID, other | 3 (7.9) | 3 (13.0) | 0 (0.0) |
Antibody deficiency w/near normal immunoglobulins | 5 (13.2) | 3 (13.0) | 2 (13.3) |
Hereditary hypogammaglobulinemia | 2 (5.3) | 1 (4.3) | 1 (6.7) |
Selective IgG deficiency | 1 (2.6) | 0 (0.0) | 1 (6.7) |
Immune deficiency w/increased IgM | 1 (2.6) | 1 (4.3) | 0 (0.0) |
HyperIgE syndrome | 1 (2.6) | 1 (4.3) | 0 (0.0) |
Immunodeficiency w/predominantly antibody defect | 1 (2.6) | 1 (4.3) | 0 (0.0) |
Prior IG therapy, n (%) | |||
IVIG | 14 (36.9) | 9 (39.1) | 5 (33.3) |
SCIG | 19 (50.0) | 12 (52.2) | 7 (46.7) |
No prior IG therapy | 5 (13.2) | 2 (8.7) | 3 (2.0) |
Treatment phase variation,an (%) | |||
Ramp-up | 37 (97.4) | 22 (95.7) | 15 (100.0) |
Maintenance regimenb | 17 (44.7) | 6 (26.1) | 11 (73.3) |
No variationc | 1 (2.6) | 1 (4.3) | 0 (0.0) |
aVariation from recommendation in package insert
bAll patients with maintenance variations also had a variation during ramp-up and are included in both variation categories
cOne patient was included because of age < 16 years but had no treatment variations