Table 2.
Summary of complement genetic profiles of women with p-aHUS and non-p-aHUS
| Women with p-aHUS (n = 51) | Women with non-p-aHUS (n = 397)a | |||||
|---|---|---|---|---|---|---|
| Eculizumab treated (n = 27) |
Not treated with eculizumab (n = 24) |
All patients (n = 51) |
Eculizumab treated (n = 187) |
Not treated with eculizumab (n = 197) |
All patients (n = 397) |
|
| Any pathogenic variant, n (%) | 10 (37.0) | 13 (54.2) | 23 (45.1) | 68 (36.4) | 89 (44.9) | 159 (40.1) |
| Anti-CFH antibody positive, n (%)a | 1 (3.7) | 2 (8.3) | 3 (5.9) | 16 (8.6) | 17 (8.6) | 33 (8.3) |
| Any pathogenic variant or anti-CFH antibody positive, n (%) | 10 (37.0) | 13 (54.2) | 23 (45.1) | 75 (40.1) | 93 (47.0) | 170 (42.8) |
| Tested for ≥ 5 pathogenic variants, no mutation identified, n (%) | 11 (40.7) | 3 (12.5) | 14 (27.5) | 61 (32.6) | 51 (25.8) | 119 (30.0) |
| Tested for < 5 pathogenic variants, no mutation identified, n (%) | 1 (3.7) | 2 (8.3) | 3 (5.9) | 8 (4.3) | 14 (7.1) | 23 (5.8) |
aHUS atypical hemolytic uremic syndrome, CNS central nervous system, CFH complement factor H, p-aHUS pregnancy-triggered aHUS
aIncludes 10 women with missing information on initial eculizumab dose and 2 women with unknown eculizumab treatment status