Frequency of CFH autoantibodies with CFHR1 and CFHR3 deficiency in the Jena cohort
. | No. . | CFH autoantibodies, no. (%) . | CFHR1/CFHR3 deficiency, no. (%) . |
---|---|---|---|
aHUS patients | 147 | 16 (11) | 22 (15) |
Controls | 100 | 0 (0) | 2 (2) |
. | No. . | CFH autoantibodies, no. (%) . | CFHR1/CFHR3 deficiency, no. (%) . |
---|---|---|---|
aHUS patients | 147 | 16 (11) | 22 (15) |
Controls | 100 | 0 (0) | 2 (2) |
The 16 patients who developed CFH autoantibodies either lack CFHR1/CFHR3 completely in plasma (n = 14) or show extremely low levels of the 2 CFHR proteins (n = 2) as determined by Western blotting. The CFHR1- and CFHR3-deficient group includes the 16 patients of the CFH autoantibody–positive group and 6 deficient patients who have no autoantibodies to CFH. No CFH autoantibodies were detected in the control group representing 100 healthy individuals. The mean absorbance of all 100 control probes was OD 0.17 (± 0.1). The highest value determined for one sample of the control group was 0.35 OD; therefore, the cutoff for false positive was set to 0.35 OD.