Table 1.
The effects of therapeutic complement inhibition in patients with TMA and coexisting hypertensive emergency or pregnancy. Single case reports have not been included.
| Presentation | Genotyping | Outcome at 12 Months | |||||||
|---|---|---|---|---|---|---|---|---|---|
| Eculizumab, n/N | Creatinine, mg/dL | Dialysis (%) | Rare Variants (%) | Pathogenic (%) | Renal Response (%) | ESKD (%) | Death (%) | ESKD in Untreated Patients | |
| Hypertensive emergency | |||||||||
| Combined data | 29/122 | Unknown | Unknown | 14 (48) | Unknown | 21 (72) | 7 (24) | 1 (3) | |
| Cavero et al. [42] | 9/19 | 8 (IQR, 7–9) | 8 (89) | 5 (56) | 3 (33) | 7 (78) | 2 (22) | 0 | 60% at 1 year (N = 10) |
| El Karoui et al. [41] | 13/76 | Unknown | Unknown | 7 (54) | Unknown | 9 (69) | 4 (31) | 0 | 64% at 1 year (N = 61) |
| Timmermans et al. [8] | 7/26 | 7 (IQR, 4–9) | 4 (57) | 2 (29) | 2 (29) | 5 (71) | 1 (14) | 1 (14) | 75% at 1 year (N = 16) a |
| Pregnancy-associated atypical HUS | |||||||||
| Combined data | 17/116 | Unknown | Unknown | 7 (41) | Unknown | 15 (88) | 2 (25) | 0 | |
| Bruel et al. [9] | 4/87 | Unknown | Unknown | 2 (50) | Unknown | 3 (75) | 1 (25) | 0 | 49% at last follow-up (N = 71) |
| Huerta et al. [48] | 10/22 | 4 (IQR, 3–5) | 3 (30) | 4 (40) | 4 (40) | 10 (100) | 0 | 0 | 55% at last follow-up (N = 11) |
| Timmermans et al. [49] | 3/7 | 5 (IQR, 4–6) | 3 (100) | 1 (33) | 0 | 2 (67) | 1 (33) | 0 | 50% at last follow-up (N = 4) |
a Patients with follow-up <12 months were excluded. HUS, hemolytic uremic syndrome. ESKD, end-stage kidney disease. IQR, inter quartile range.