Table 1.
Demographic, clinical, and some laboratory findings of patients.
| CVID (n = 40) n (%) |
Selective IgAD (n = 36) | Partial IgAD (n = 34) | IgAD (n = 70) | P | Familial cases
(n = 37) n (%) |
Sporadic cases
(n = 73) n (%) |
P | |
|---|---|---|---|---|---|---|---|---|
| Gender (female/male) | 11/29 | 14/22 | 15/19 | 29/41 | 0.314 | 11/26 | 29/44 | 0.303 |
| Age (months) | 161.7 ± 59.2 | 120.8 ± 49.1 | 110.8 ± 41.6 | 116.1 ± 45.6 | 0.000 | 136.3 ± 64.9 | 131.1 ± 50.1 | 0.648 |
| Age at the beginning of symptoms (months) | 46.1 ± 54.2 | 22.5 ± 23.2 | 30.4 ± 42.1 | 26.4 ± 23.1 | 0.166 | 34.6 ± 43.4 | 32.9 ± 35.3 | 0.828 |
| Age at diagnosis (months) | 97.5 ± 51.2 | 65.2 ± 44.7 | 69.1 ± 42.1 | 67.1 ± 43.2 | 0.009 | 73.6 ± 53.3 | 80.1 ± 45.7 | 0.482 |
| Parental consanguinity | 16 (41%) | 3 (8.8%) | 2 (5.9%) | 5 (7.1%) | 0.000 | 5 (13.5%) | 16 (21.9%) | 0.212 |
| Family history | 6 (15.2%) | 0 | 7 (20.6%) | 7 (10%) | 0.003 | 5 (13.5%) | 7 (9.9%) | 0.392 |
| Number of familial/sporadic cases | 13/27 | 10/26 | 14/20 | 24/46 | 0.489 | Selective IgAD n = 10 Partial IgAD n = 14 CVID n = 13 |
Selective IgAD n = 26 Partial IgAD n = 20 CVID n = 27 |
0.486 |
| Symptoms | ||||||||
| R. URTI | 16 (42.1%) | 34 (94.3%) | 28 (82.4%) | 62 (88.1%) | 0.000 | 21 (56.7%) | 59 (80.8%) | 0.021 |
| R. LRTI | 17 (44.7%) | 2 (5.7%) | 6 (17.6%) | 8 (11.4%) | 13 (35.1%) | 12 (16.4%) | ||
| Recurrent or chronic diarrhea | 2 (5.3%) | (–) | (–) | (–) | 2 (5.4%) | − | ||
| Other (failure to thrive, autoimmune hemolytic anemia, chronic arthritis, malignancy) | 3 (7.9%) | (–) | (–) | (–) | 1 (2.7%) | 2 (2.7%) | ||
| Immunoglobulin levels (mg/dL) | ||||||||
| IgG | 446.1 ± 138.6 | 1442.5 ± 509.2 | 1010.5 ± 325.9 | 1132.2 ± 479.1 | 0.340 | 869.1 ± 472.7 | 993.4 ± 577.2 | 0.232 |
| IgM | 62.7 ± 59.8 | 110.2 ± 79.6 | 103.1 ± 38.1 | 106.8 ± 62.6 | 0.270 | 73.3 ± 51.0 | 100.1 ± 69.5 | 0.040 |
| IgA | 25.7 ± 23.2 | < 6 | 28.7 ± 16.3 | 17.1 ± 15.9 | 0.000 | 18.5 ± 15.7 | 29.7 ± 36.5 | 0.078 |
| Autoantibody positivity | ||||||||
| Anti-nuclear antibody | 9 (28.1%) | 11 (30.6) | 5 (14.7%) | 16 (22.1%) | 0.342 | 7 (18.9%) | 18 (24.6%) | 0.307 |
| Direct coombs test | 6 (15%) | 1 (2.8%) | 1 (2.9%) | 2 (2.8%) | 0.013 | 1 (2.7%) | 7 (9.6%) | 0.121 |
| Anti-thyroglobulin antibody | 1 (5.3%) | 2 (5.6%) | 2 (5.9%) | 4 (5.7%) | 0.976 | 0 | 5 (6.8%) | 0.021 |
| Anti-microsomal antibody | 1 (5.3%) | 6 (16.6%) | 4 (11.7%) | 10 (14.2%) | 0.112 | 0 | 10 (13.6%) | 0.013 |
| Anti-endomysium antibody | (–) | 1 (2.8%) | (–) | 1 (1.4%) | 0.440 | 0 | 1 (1.4%) | 0.439 |
| Rheumatic factor | 1 (2.5%) | 1 (2.8%) | (–) | 1 (1.4%) | 0.579 | 1 (2.7%) | 1 (1.4%) | 0.736 |
CVID, common variable immunodeficiency; IgAD, immunoglobulin A deficiency; R. URTI: recurrent upper respiratory tract infections; R. LRTI: recurrent lower respiratory tract infections.