Table 1.
Clinical characteristics and immunological data of 47 CVIDs patients.
| All CVIDs n=47 |
Group 1 (S1 IgG+/S+ and S++ MBCs+) n=14 (30%) |
Group 2 (S1 IgG+/S+ MBCs+) n=7 (15%) |
Group 3 (S1 IgG+/S+ MBCs-) n=9 (19%) |
Group 4 (S1 IgG-/S+ MBC-) n= 17 (36%) |
p values <0.05 | |
|---|---|---|---|---|---|---|
| Sex. Male n (%) | 28 (59.6) | 8 (57) | 4 (57) | 6 (66.7) | 10 (58.8) | – |
| Age. median (IQR) | 53 (45-66) | 54 (46-71) | 53 (46-59) | 45 (38-60) | 56 (50-65) | – |
| Age at IEI diagnosis median (IQR) |
38 (32-51) | 40 (35-57) | 37 (29.5-49) | 39 (30-44) | 38 (33-51) | – |
| Serum IgG at IEI diagnosis median (IQR) |
307 (166-433) | 440 (456-583) | 144 (28-259) |
320 (120-396) | 240 (166-375) | gr 1 vs 4 p<0.0001 gr 1 vs 3 p=0.002 gr 1 vs 2 p=0.001 |
| Serum IgA at IEI diagnosis median (IQR) |
20 (6-28) | 47 (6-103) | 13 (8-16) | 25 (16-26) | 11 (1-27) | gr 1 vs 4 p=0.0018 gr 1 vs 3 p=0.0052 gr 2 vs 4 p=0.0489 gr 2 vs 3 p=0.0428 |
| Serum IgM at IEI diagnosis median (IQR) |
17 (3-42) | 50 (25-59) | 18 (3-52) | 16 (3-36) | 5 (0-17) | gr 1 vs 4 p<0.0001 gr 2 vs 4 p=0.0238 |
| Persistent lymphopenia (<1000 cell/mm3), n (%) |
10 (21.3) | 0 | 0 | 4 (44) | 6 (35.3) | gr 1 vs 4 p=0.013 gr 1 vs 3 p=0.006 gr 2 vs 3 p=0.042 |
| Dysregulatory- phenotype, n (%) |
22 (46.8) | 4 (28.6) | 2 (27.6) | 5 (55.6) | 11 (64.7) | gr 1 vs 4 p=0.049 |
| Autoimmunity, n (%) |
16 (34.0) | 1 (7) | 2 (29) | 5 (55.5) | 8 (47) | gr 1 vs 4 p=0.015 gr 1 vs 3 p=0.010 |
| Autoimmune cytopenias, n (%) |
9 (19.1) | 0 (0) | 1 (14) | 2 (22) | 6 (35) | gr 1 vs 4 p=0.013 |
| Lymphoma, n (%) |
4 (8.5) | 2 (14) | 0 | 0 | 2 (12) | – |
| Chronic Lung Disease, n (%) |
19 (41.3) | 3 (21) | 1 (14) | 5 (55) | 10 (62) | gr 1 vs 4 p=0.024 gr 2 vs 4 p=0.033 |
| Immunosuppressive treatment, n (%) |
6 (12.8) | 0 | 1 (14) | 0 | 5 (29.4) | gr 1 vs 4 p=0.027 |
| CVIDs - related manifestations, median (IQR) |
1 (0-2) | 0 (0-1) | 1 (0-1) | 2 (1-3) | 2 (1-3) | gr 1 vs 4 p<0.0001 gr 1 vs 3 p<0.0001 gr 2 vs 3 p=0.014 gr 2 vs 4 p=0.005 |
CVIDs, Common Variable Immune Deficiencies; IEIs, Inborn Errors of Immunit; IQR, interquartile range; MBC, Memory B Cells; n, number.