Table 2.
Summary of clinical outcome in patients with aHUS
| Genetic defect | Frequency | Response to short-term plasma therapy | Long-term outcome | Outcome after kidney transplantation |
|---|---|---|---|---|
| CFH | 20–30% | Rate of remission: 60% (dose- and timing-dependent) | Rate of death or ESRD: 70–80% | Rate of recurrence: 80–90% |
| CFI | 4–10% | Rate of remission: 30–40% | Rate of death or ESRD: 60–80% | Rate of recurrence: 80–90% |
| CFHR1 & 3 with CFH autoantibodies | 6% | Rate of remission: 70–80% | Rate of death or ESRD: 30–40% | Rate of recurrence: 20% |
| MCP | 10–15% | No indication for therapy | Rate of ESRD or death: <20% | Rate of recurrence: 15–20% |
| CFB | 1–2% | Rate of remission: 30% | Rate of death or ESRD: 70% | Recurrence in one case |
| C3 | 5–10% | Rate of remission: 40–50% | Rate of death or ESRD: 60% | Rate of recurrence: 40–50% |
| THBD | 5% | Rate of remission: 60% | Rate of death or ESRD: 60% | Recurrence in one case |