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Abstract: PUB034

Escherichia coli-Associated Hemolytic Uremic Syndrome (HUS) Treated with Eculizumab

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Author

  • Gipson, Graham Thomas, Virginia Commonwealth University School of Medicine, Richmond, Virginia, United States
Introduction

Eculizumab, a terminal complement inhibitor, is enjoying use in a number of complement-disregulatory disease. A major use of eculizumab is treatment of atypical hemolytic–uremic syndrome (aHUS). Since Shiga toxin–associated HUS (STAHUS) are driven by toxic complement dysregulation, it stands to reason that eculizumab might provide therapeutic benefit in STAHUS.

Case Description

A 41-year old white male presented with a 2-day history of abdominal pain and frankly blood diarrhea. The only antecedent event of note was consumption of sushi. Initial exam showed normal vital signs and bilateral lower abdominal tenderness without signs of peritonitis. He received empiric antibiotics (ciprofloxacin and metronidazole) along with supportive care. Over 24 h he developed hypoactive encephalopathy, severe azotemia with oliguria, and a sharp drop in hemoglobin and platelet count. After 96 h his stool culture disclosed E. coli O157:H7, thus prompting the diagnosis of E. coli O157:H7–associated hemorrhagic enterocolitis with probable neurologically significant HUS. He underwent one session of plasma exchange followed by a single 900-mg dose of eculizumab. Continuous hemodialysis was ultimately initiated for oliguric AKI. After almost 3 weeks in-hospital he enjoyed a near-total recovery and was discharged to a rehabilitation facility prior to return home. The chronology of key clinical events and laboratory parameters is shown in the figure.

Discussion

We demonstrate here the favorable clinical course of Shiga toxin–associated hemolytic–uremic syndrome (STAHUS) following a single dose of the terminal complement inhibitor eculizumab in an otherwise healthy adult patient. Successful cases like this add to the base of evidence that eculizumab is useful in treating multiple diseases driven by complement system dysregulation.