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Abstract: SA-PO384

(STEC-HUS) in Adults: An Underrecognized Cause of Thrombotic Microangiopathy

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Duineveld, Caroline, Radboudumc, Nijmegen, Netherlands
  • Wijnsma, Kioa L., Amalia Children's Hospital, Nijmegen, Netherlands
  • Wetzels, Jack F., Radboudumc, Nijmegen, Netherlands
  • Van De Kar, Nicole, Amalia Children's Hospital, Nijmegen, Netherlands

Group or Team Name

  • the Dutch aHUS working group
Background

In children, most cases of hemolytic uremic syndrome (HUS) are caused by an infection with a Shiga toxin-producing Escherichia coli (STEC). In contrast, in adults most cases of HUS are considered to be atypical HUS (aHUS), attributed to dysregulation of the alternative complement system. However, during major outbreaks of STEC infection, hemolytic uremic syndrome in adults is not uncommon and morbidity and mortality, especially in elderly patients, may be considerable. Only few sporadic cases of STEC-HUS in adults have been described. Typically, STEC HUS is preceded by diarrhea, which is reported to be bloody in 60% of the patients. In the Netherlands routine screening for STEC is not recommended in adults with TMA, unless diarrhea is present.

Methods

Since 2015/2016, patients presenting with TMA in the Netherlands, in which aHUS is suspected, are discussed by the Dutch aHUS working group. Diagnostic options are carefully evaluated. This has increased awareness for STEC-HUS testing.

Results

Of 44 TMA patients who were discussed in the Dutch aHUS Working Group in the time period 2015/2016-2018, aHUS was diagnosed in 24 patients. STEC-HUS was found as the cause of TMA in 10 adult patients. Detailed clinical information could be obtained from seven STEC-HUS patients. The age of the patients ranged between 17 and 74 years. Of note, in three patients no diarrhea was reported. All presented with TMA (median thrombocytes 30 x 109/L (range 8-33), LDH 1409 g/L (range 946-3265) and an undetectable haptoglobin (<0.10 g/L)). Fecal or rectal swab PCR for Shiga toxin (Stx) was positive in all patients. In five patients PCR Stx2 was positive; two of them also had a positive PCR for STx1. Serotype could be identified in four patients: O157, O27:H6, O-:H34 and O183:H18. Acute renal injury was seen in six patients, and four were treated with hemodialysis. Renal function recovered in five patients, however one patient remaining dependent on dialysis. Median eGFR was 83 ml/min/1.73 m2 (range <5-133) after a follow-up of 1 to 15 months after presentation.

Conclusion

STEC HUS among adults is not uncommon and may not be preceded by diarrhea. We advocate routine screening for STEC infection in adults patients presenting with TMA.

Funding

  • Commercial Support