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Hypertensive emergency and hemolytic uremic syndrome
or a rare complement variant disclosed major differences in renal survival: patients without either HE or comple- ment variants had a significantly better outcome than all other groups (with HE, and/or rare complement variants) (Figure 5A). Five-year renal survival rates were 77% (no HE and no complement variant), 22% (HE without a complement variant), 25% (complement variant without HE) and 23% (HE with a complement variant) (P=0.02) (Figure 5A). The median serum creatinine and estimated glomerular filtration rate of patients without either HE or
A
a complement variant at last follow-up were 75 μM and 82 mL/min/1.73 m2, respectively. No difference in out- come was observed according to the type of complement variant (data not shown). Similar results were obtained when analyzing the whole cohort, including patients treated with eculizumab, since 5-year renal survival rates were 84% (no HE and no complement variant), 21% (HE without a complement variant), 44% (complement vari- ant without HE) and 34% (HE with a complement vari- ant) (P=0.001) (Figure 5B).
B
C
Figure 4. Renal survival of patients treated or not with eculizumab. Analysis of renal survival without end-stage renal disease or death according to use of eculizumab treatment in: (A) the whole cohort, P<0.001; (B) in patients without hypertensive emergency (HE) and atyp- ical hemolytic uremic syndrome, P<0.001; and (C) in HE-aHUS patients, P=0.18. Log-rank test. Follow-up was not available for two patients with HE-aHUS.
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