Shiga Toxin-Associated Hemolytic Uremic Syndrome in Adults, France, 2009-2017

Abstract

We conducted a retrospective study on hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli (STEC) in 96 adults enrolled in the cohort of the National Reference Center for Thrombotic Microangiopathies network in France during 2009-2017. Most infections were caused by STEC strains not belonging to the O157 or O104 serogroups. Thirty (31.3%) patients had multiple risk factors for thrombotic microangiopathy. In total, 61 (63.5%) patients required dialysis, 50 (52.1%) had a serious neurologic complication, 34 (35.4%) required mechanical ventilation, and 19 (19.8%) died during hospitalization. We used multivariate analysis to determine that the greatest risk factors for death were underlying immunodeficiency (hazard ratio 3.54) and severe neurologic events (hazard ratio 3.40). According to multivariate analysis and propensity score-matching, eculizumab treatment was not associated with survival. We found that underlying conditions, especially immunodeficiency, are strongly associated with decreased survival in adults who have hemolytic uremic syndrome caused by STEC.

Keywords: E. coli; Escherichia coli; France; HUS; STEC; Shiga toxin; Shiga toxin–producing Escherichia coli; bacteria; enteric infections; food safety; food-borne infections; hemolytic uremic syndrome; thrombotic microangiopathy.

Figure 1

Distribution of adults with Shiga toxin–associated hemolytic uremic syndrome, France, 2009–2017. A) Geographic distribution of cases and thrombotic microangiopathy reference centers. The Centre National de Référence des Microangiopathies Thrombotiques is a national network comprising 1 coordination center, 5 constitutive centers, and 21 competence centers. B) Age and sex distribution of cases. C) Bimonthly distribution of cases according to serogroup. Of patients with minor serogroups, 4 had strains belonging to O106, 3 to O128, 3 to O174, 2 to O113, 1 to O100, 1 to O126, 1 to O148, 1 to O177, 1 to O78, 1 to O84, and 7 to an O serogroup not typable at the time of identification. ND, not determined.

Figure 2

Kaplan-Meier survival plots of adults with Shiga toxin–associated hemolytic uremic syndrome, France, 2009–2017. A) Overall. B) By age-weighted Charlson comorbidity index. C) By STEC serogroup. D) By treatment. Plots show time from admission to death. p values determined using log-rank test. BSC, best standard of care; CCI, age-weighted Charlson comorbidity index; ECZ, eculizumab; TPE, therapeutic plasma exchange.