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Hypertensive emergency and hemolytic uremic syndrome
Of these, 76 (54%) had concomitant HE (HE-aHUS), and 61 did not have HE (noHE-aHUS) (Figure 1). A total of 7/44 noHE-aHUS females, and 1/32 HE-aHUS females were diagnosed after pregnancy. Eculizumab was used in 13/76 (17%) HE-aHUS and 17/61 (28%) noHE-aHUS patients. The median follow-up was 39.9 months, and 57 patients presented with definitive end-stage renal disease at onset. Follow-up was not available for two patients with HE-aHUS.
The patients’ clinical and biological characteristics are presented in Table 1. The male/female ratio of the 76 patients with HE-aHUS was 44/32 (male 58%). The patients’ mean age was 37 years, and their mean sys- tolic/diastolic blood pressure was 214/128 mmHg. The mean hemoglobin concentration was 8.5 g/dL and throm- bocytopenia was profound [mean 104 x 109/L; platelet count <100 x 109/L in 42% (32 patients)]. Acute kidney injury was severe with 81% patients requiring dialysis at onset. Twelve of the 76 patients (16%) presented with a diagnosis of long-lasting high blood pressure or left ven- tricular hypertrophy. Kidney biopsy, performed in 24 HE- aHUS patients (32%), showed typical features of throm- botic microangiopathy with arteriolar thromboses, except in one patient with only glomerular retraction sug- gestive of glomerular ischemia. The patients with HE- aHUS had a severe prognosis, since 1-year and 5-year
renal survival rates were 36% and 23%, respectively, in patients not treated with eculizumab (Figure 2).
Compared to patients with noHE-aHUS (Table 1), HE- aHUS patients were more frequently males (58% vs. 28%, P<0.01) with a significantly higher frequency of neurological involvement and higher hemoglobin levels (8.5 vs. 7.5 g/dL, P=0.01). No significant difference was observed in age, cardiac involvement, platelet count, pres- ence of elevated lactate dehydrogenase, schistocyte fre- quency or dialysis requirement at onset between the two groups (Table 1).
Four deaths occurred among the 76 HE-aHUS patients during follow-up, whereas there were five deaths among the 61 noHE-aHUS patients (5.2% vs. 8.2% respectively, P=0.5). The deaths were related to sepsis (3 patients), a cardiac etiology (2 patients), cancer (1 patient), cerebral hematoma (1 patient), and to an unknown cause (2 patients). Five of these nine patients already had end- stage renal disease before death. Without eculizumab treatment, the prognosis of HE-aHUS (n=61) and noHE- aHUS (n=44) patients was similar, since 1-year and 5-year renal survival rates were 36% vs. 45%, and 23% vs. 40% in the HE-aHUS and noHE-aHUS patients, respectively (P=0.1) (Figure 2). However, in the whole cohort includ- ing patients treated with eculizumab, those with HE- aHUS had a worse renal prognosis than those with noHE-
Figure 1. Study flow chart. Flow chart for the inclusion criteria of patients within the adult population of the French HUS registry screened for genetic abnormalities (n=405). A total of 137 patients were eligible for enrollment in the study. HUS: hemolytic uremic syndrome; HE: hypertensive emergency; aHUS: atypical hemolytic uremic syndrome.
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