Introduction
Methods
Population Analyzed
Study Design
Questionnaires
Statistical Analysis
Results
Patient Population and Immunoglobulin Administration
fSCIG n = 33 | SCIG n = 59 | IVIG n = 206 | SCIG + IVIG n = 6 | |
---|---|---|---|---|
Age (years), mean (SD) | 40.3 (11.2)c | 45.2 (13.1) | 49.2 (16.1)a | 39.2 (13.1) |
Sex (females), n (%) | 11.5(33.1) | 26.1 (44.2) | 113.2 (55.2) | 4.5 (67.2) |
Occupation (employed), n (%) | 25 (75) | 34 (57) | 99 (48) | 3 (50) |
Education (≥ high school), n (%) | 30 (90) | 48 (81) | 150 (73) | 5 (83) |
Time (years) from PID diagnosis, mean (SD) | 11.2 (9.2) | 9.0 (7.1)d | 12.3 (11.1) | 19.1 (11.0)b |
Ig serum levels at diagnosis: | ||||
- IgG (mg/dL), mean (SD) | 326.3 (183.5) | 313.3 (161.2) | 246.5 (179.2) | 160.2 (186.3) |
- IgM (mg/dL), mean (SD) | 23.2 (21.2) | 27.2 (24.6) | 26.1 (38.6) | 5.2 (5.1) |
- IgA (mg/dL), mean (SD) | 39.1 (52.3) | 28.2 (33.5) | 22.3 (58.3) | 18.2 (26.2) |
IgG trough serum levels (mg/dL), mean (SD) | 713.3 (115.1) | 745.0 (109.2)d | 725.1 (180.2) | 600.5 (122.3)b |
CD3+ CD4+/mm3, mean (SD) | 785.3 (542.1) | 740 (381) | 624.1 (343.2) | 935.7 (763.1) |
CD19+ (%), mean (SD) | 8.6 (8.3) | 12 (17) | 13.1 (23.2) | 6.1 (1.0) |
CD19+ CD27+ IgM− IgD− (%), mean (SD) | 7.8 (14.7) | 7 (10) | 6.1 (14.2) | 2.1 (3.2) |
fSCIG n = 33 | SCIG n = 59 | IVIG n = 206 | SCIG + IVIG n = 6 | |
---|---|---|---|---|
IgG trough serum levels (mg/dL), mean (SD) | 723.5 (143.1) | 797 (159) | 756.2 (214.2) | 607.3 (162.1) |
IgM serum levels (mg/dL), mean (SD) | 28.6 (25.2) | 23 (26) | 30.1 (51.2) | 10.5 (9.5) |
IgA serum levels (mg/dL), mean (SD) | 40.2 (58.2) | 30 (45) | 24.1 (63.2) | 12.3 (24.4) |
Cumulative monthly Ig dose (mg/kg), mean (SD) | 317.8 (120.3)d | 359.0 (152.5) | 351.5 (148.6) | 490.2 (238.1)a |
Number of monthly administrations mean (SD) | 2.1 (1.0)b | 4.1 (1.3)a,c | 2.0 (1.0)b | 4.2 (2.5) |
Antibiotic prophylaxis, n (%) | 4 (12.1) | 9 (15.2) | 23 (11.5) | 2 (33.2) |
Adherent to treatment, n (%) | 31 (93.9) | 54 (91.5) | 194 (94.4) | 5 (83.3) |
CVID-Associated Morbidities
FSCIG n = 33 | SCIG n = 59 | IVIG n = 206 | SCIG + IVIG n = 6 | |
---|---|---|---|---|
Diarrhea, episodes-year, mean (SD) | 4.1 (2.7) | 3.2 (3.1) | 3.3 (2.8) | 4.7 (3.9) |
Sinusitis, episodes-year, mean (SD) | 1.6 (1.6) | 1.6 (1.6) | 1.7 (1.6) | 2.7 (1.8) |
Bronchitis, episodes-year, mean (SD) | 1.3 (1.5) | 1.4 (1.5) | 1.3 (1.5) | 1.7 (1.6) |
Otitis, episodes-year, mean (SD) | 0.3 (0.8) | 0.3 (0.8) | 0.4 (1.0) | 0.3 (0.8) |
Patients with pneumonia, n (%) | 6 (18.8) | 5 (8.9) | 30 (14.9) | 1 (16.7) |
All infections requiring treatment episodes-year, mean (SD) | 4.4 (5.2)§ | 3.9 (3.8) | 3.8 (4.0) | 9.0 (6.1)§ |
Hospital admission (all causes), n (%) | 2 (6.1) | 7 (11.9) | 15 (7.3) | 1 (16.7) |
Quality of Life
Q.5 I had to give up making long-term plans | Q.9 I had discomfort and/or pain in my joints | Q.11 I was afraid I would run out of medication | Q.12 I was afraid of adverse reaction to Ig therapy | Q.15 I felt less independent than usual | Q.24 Ig therapy bothered me | Q.31 I felt troubled by relationship with other patients | Q32 I felt tired | ||
---|---|---|---|---|---|---|---|---|---|
Age | p value | 0.314 | < 0.0001 | 0.191 | 0.618 | 0.011 | 0.120 | 0.003 | 0.242 |
≥ 40 year | Mean (SD) | 1.5 (1.4) | 1.9 (1.3) | 0.7 (1.0) | 0.7 (0.9) | 1.1 (1.1) | 0.7 (0.9) | 0.4 (0.8) | 2.2 (1.2) |
< 40 year | Mean (SD) | 1.4 (1.2) | 1.3 (1.2) | 0.6 (0.9) | 0.6 (0.9) | 0.8 (1.0) | 0.9 (1.0) | 0.1 (0.4) | 2.0 (1.2) |
Sex | p value | 0.001 | 0.009 | 0.881 | 0.449 | 0.007 | 0.221 | 0.112 | < 0.0001 |
Female | Mean (SD) | 1.7 (1.3) | 1.9 (1.3) | 0.7 (1.0) | 0.7 (0.9) | 1.2 (1.1) | 0.8 (1.0) | 0.4 (0.8) | 2.4 (1.2) |
Male | Mean (SD) | 1.2 (1.3) | 1.5 (1.2) | 0.7 (1.0) | 0.6 (1.0) | 0.9 (1.0) | 0.7 (0.9) | 0.2 (0.5) | 1.8 (1.2) |
COPD | p value | 0.011 | 0.066 | 0.056 | 1.000 | 0.013 | 0.704 | 0.216 | 0.004 |
Yes | Mean (SD) | 1.9 (1.3) | 2.0 (1.3) | 0.9 (1.2) | 0.7 (1.0) | 1.4 (1.2) | 0.8 (1.0) | 0.4 (0.7) | 2.5 (1.1) |
No | Mean (SD) | 1.4 (1.3) | 1.7 (1.3) | 0.7 (0.9) | 0.7 (0.9) | 0.9 (1.0) | 0.8 (0.9) | 0.3 (0.7) | 2.0 (1.2) |
Enteropathy | p value | 0.046 | 0.140 | 0.512 | 0.289 | 0.106 | 0.408 | 0.986 | 0.128 |
Yes | Mean (SD) | 1.8 (1.3) | 1.9 (1.0) | 0.6 (0.9) | 0.8 (1.1) | 1.2 (1.2) | 0.8 (1.0) | 0.3 (0.7) | 2.3 (1.2) |
No | Mean (SD) | 1.4 (1.3) | 1.7 (1.4) | 0.7 (1.0) | 0.6 (0.9) | 1.0 (1.0) | 0.7 (0.9) | 0.3 (0.7) | 2.1 (1.2) |
Autoimmunity | p value | 0.173 | 0.080 | 0.615 | 0.747 | 0.523 | 0.079 | 0.586 | 0.123 |
Yes | Mean (SD) | 1.6 (1.3) | 1.9 (1.2) | 0.6 (1.0) | 0.6 (0.9) | 1.1 (1.1) | 0.9 (1.0) | 0.3 (0.6) | 2.3 (1.1) |
No | Mean (SD) | 1.4 (1.3) | 1.7 (1.3) | 0.7 (1.0) | 0.7 (0.9) | 1.0 (1.1) | 0.7 (0.9) | 0.3 (0.7) | 2.1 (1.2) |
Education | p value | 0.013 | < 0.0001 | 0.131 | 0.103 | 0.078 | 0.382 | 0.197 | 0.257 |
≥ High school | Mean (SD) | 1.4 (1.3) | 1.6 (1.3) | 0.7 (0.9) | 0.6 (0.9) | 1.0 (1.1) | 0.7 (1.0) | 0.3 (0.6) | 2.1 (1.2) |
< High school | Mean (SD) | 1.8 (1.4) | 2.2 (1.2) | 0.9 (1.2) | 0.8 (0.9) | 1.2 (1.2) | 0.9 (0.9) | 0.4 (0.8) | 2.3 (1.3) |
Occupation | p value | 0.002 | 0.001 | 0.401 | 0.048 | 0.001 | 0.819 | 0.307 | 0.013 |
Employed | Mean (SD) | 1.2 (1.3) | 1.5 (1.3) | 0.7 (1.0) | 0.6 (0.8) | 0.8 (1.0) | 0.8 (1.0) | 0.3 (0.6) | 2.0 (1.2) |
Unemployed | Mean (SD) | 1.7 (1.4) | 2.0 (1.3) | 0.8 (1.0) | 0.8 (1.0) | 1.3 (1.1) | 0.8 (0.9) | 0.3 (0.8) | 2.3 (1.2) |
GHQ status | p value | < 0.0001 | < 0.0001 | 0.126 | 0.002 | < 0.0001 | < 0.0001 | 0.002 | < 0.0001 |
Positive | Mean (SD) | 1.9 (1.3) | 2.1 (1.3) | 0.8 (1.0) | 0.9 (1.0) | 1.4 (1.1) | 1.0 (1.0) | 0.5 (0.9) | 2.7 (1.0) |
Negative | Mean (SD) | 1.0 (1.2) | 1.4 (1.2) | 0.6 (0.9) | 0.5 (0.8) | 0.6 (0.9) | 0.6 (0.8) | 0.2 (0.5) | 1.7 (1.1) |
Standardized beta | p value | |
---|---|---|
Q5. I had to give up making long-term plans | ||
Route of IgRT | − 0.082 | 0.202 |
GHQ positive status | 0.297 | < 0.0001 |
Age | 0.033 | 0.613 |
Sex | 0.101 | 0.121 |
COPD | 0.068 | 0.276 |
Enteropathy | 0.111 | 0.075 |
Education | − 0.103 | 0.132 |
Occupation | − 0.063 | 0.366 |
Q9. I had discomfort and/or pain in my joints | ||
Route of IgRT | 0.075 | 0.241 |
GHQ positive status | 0.196 | 0.003 |
Age | 0.189 | 0.005 |
Sex | 0.045 | 0.492 |
COPD | 0.022 | 0.729 |
Enteropathy | 0.050 | 0.421 |
Education | − 0.115 | 0.094 |
Occupation | − 0.072 | 0.308 |
Q11. I was afraid I would run out of medication | ||
Route of IgRT | − 0.073 | 0.289 |
GHQ positive status | 0.16 | 0.022 |
Age | 0.075 | 0.289 |
Sex | − 0.032 | 0.649 |
COPD | 0.135 | 0.045 |
Enteropathy | − 0.036 | 0.591 |
Education | 0.001 | 0.994 |
Occupation | − 0.034 | 0.653 |
Q12. I was afraid of adverse reaction to Ig therapy | ||
Route of IgRT | − 0.107 | 0.115 |
GHQ positive status | 0.204 | 0.004 |
Age | 0.012 | 0.861 |
Sex | − 0.044 | 0.528 |
COPD | 0.007 | 0.922 |
Enteropathy | 0.038 | 0.568 |
Education | − 0.133 | 0.067 |
Occupation | − 0.051 | 0.494 |
Q15. I felt less independent than usual | ||
Route of IgRT | − 0.042 | 0.504 |
GHQ positive status | 0.329 | < 0.0001 |
Age | 0.134 | 0.042 |
Sex | 0.065 | 0.314 |
COPD | 0.074 | 0.237 |
Enteropathy | 0.059 | 0.34 |
Education | 0.018 | 0.789 |
Occupation | − 0.077 | 0.272 |
Q24. Ig therapy bothered me | ||
Route of IgRT | − 0.13 | 0.047 |
GHQ positive status | 0.301 | < 0.0001 |
Age | − 0.191 | 0.005 |
Sex | 0.042 | 0.529 |
COPD | 0.024 | 0.708 |
Enteropathy | 0.063 | 0.325 |
Education | − 0.108 | 0.121 |
Occupation | − 0.044 | 0.534 |
Q31. I felt troubled by relationship with other patients | ||
Route of IgRT | 0.045 | 0.506 |
GHQ positive status | 0.176 | 0.012 |
Age | 0.07 | 0.320 |
Sex | 0.033 | 0.634 |
COPD | 0.02 | 0.762 |
Enteropathy | 0.005 | 0.942 |
Education | − 0.085 | 0.243 |
Occupation | 0.022 | 0.774 |
Q32. I felt tired | ||
Route of IgRT | − 0.034 | 0.583 |
GHQ positive status | 0.412 | 0.0001 |
Age | − 0.011 | 0.868 |
Sex | 0.095 | 0.134 |
COPD | 0.124 | 0.042 |
Enteropathy | − 0.005 | 0.939 |
Education | − 0.025 | 0.702 |
Occupation | − 0.027 | 0.693 |