Circulating complement profile in aHUS patients
| Complement parameters . | Disease phase . | Overall . | Mutations or anti-CFH Ab . | No mutations . |
|---|---|---|---|---|
| Reduced C3 serum levels (83-180 mg/dL)* | Acute† | 10 (18) | 5 (9) | 5 (9) |
| Remission‡ | 15 (32) | 11 (25) | 4 (7) | |
| Increased C5a plasma levels (1.9-13.1 ng/mL)* | Acute† | 9 (19) | 3 (10) | 6 (9) |
| Remission‡ | 21 (36) | 15 (27) | 6 (9) | |
| Increased SC5b-9 plasma levels (127-400 ng/mL)* | Acute† | 10 (19) | 4 (10) | 6 (9) |
| Remission‡ | 23 (36) | 20 (27) | 3 (9) |
| Complement parameters . | Disease phase . | Overall . | Mutations or anti-CFH Ab . | No mutations . |
|---|---|---|---|---|
| Reduced C3 serum levels (83-180 mg/dL)* | Acute† | 10 (18) | 5 (9) | 5 (9) |
| Remission‡ | 15 (32) | 11 (25) | 4 (7) | |
| Increased C5a plasma levels (1.9-13.1 ng/mL)* | Acute† | 9 (19) | 3 (10) | 6 (9) |
| Remission‡ | 21 (36) | 15 (27) | 6 (9) | |
| Increased SC5b-9 plasma levels (127-400 ng/mL)* | Acute† | 10 (19) | 4 (10) | 6 (9) |
| Remission‡ | 23 (36) | 20 (27) | 3 (9) |
Ab, antibody.
Limits of normal ranges (as defined in supplemental “Methods”). In parentheses are the numbers of patients for whom data were available, and outside the parentheses are the numbers of patients with reduced C3 or increased C5a or SC5b-9 levels.
One patient was receiving eculizumab at the time of the test.
Eight patients were receiving eculizumab at the time of the tests.