Table 1.
Characteristics of 38 patients with atypical hemolytic uremic syndrome before and after eculizumab discontinuation
| Patient Characteristics | Value |
|---|---|
| Age at eculizumab discontinuation, yr | 30 (2–79) |
| <18 | 9 (24) |
| ≥18 | 29 (76) |
| Complement genes variantsa | 21 (55) |
| CFHb | 11 (29) |
| MCP | 8 (21) |
| C3 | 1 (2.5) |
| CFI | 1 (2.5) |
| No variant | 16 (42.5) |
| Anti-CFH antibodies | 1 (2.5) |
| Patients with ≥1 aHUS episode before eculizumab use | 9 (23)c |
| SCr at eculizumab start, mg/dl | |
| Adults | 7.9 (1.3–19) |
| Children | 1.9 (0.2–3.9) |
| Patients requiring dialysis at eculizumab start | 18 of 38 (47)d |
| Duration of eculizumab treatment, mo | 17.5 (2–50) |
| SCr at eculizumab discontinuation, mg/dl | |
| Adults | 1 (0.7–2.8) |
| Children | 0.4 (0.25–0.46) |
| Patients with CKD | 4 (11) |
| Duration of follow-up after eculizumab discontinuation, mo | 22 (5–43) |
| Patients with aHUS relapse | 12 of 38 (32) |
| CFH | 8 of 11 (72) |
| MCP | 4 of 8 (50) |
| No variant | 0 of 16 (0) |
| Time to relapse, mo | 7.5 (3–29) |
| SCr at last follow-up, mg/dl | |
| Adults | 1 (0.7–2.8) |
| Children | 0.44 (0.25–1) |
Values are shown as n (%) or median (full range). CFH, complement factor H; MCP, membrane cofactor protein; CFI, complement factor I; aHUS, atypical hemolytic uremic syndrome; SCr, serum creatinine.
a
Variant with minor allele frequency <0.1%.
b
Including one combined CFH/CFI variants (p.Ile340Thr).
c
Patients 2, 4, 9, 12–16, and 31.
d
One child (patient 27 required dialysis).