(1462) Eculizumab for Treatment of Shiga Toxin-Producing E coli Hemolytic Uremic Syndrome (STEC-HUS)

Introduction: STEC-HUS, the most common type of HUS is one of several forms of thrombotic microangiopathies. While symptoms are typically limited to diarrhea, in rare cases STEC-HUS leads to life threatening symptoms such as seizures and encephalopathy. The initial insult of STEC-HUS is the destruction of the endothelium by Shiga toxin, which leads to downstream complement activation. Due to complement system involvement, limited data describe use of anti-complement therapy with eculizumab. However, eculizumab is costly and has the potential for severe adverse effects. Herein, we describe the rapid neurological improvement following a single dose of eculizumab in a patient with catastrophic STEC-HUS.

Description: A 36-year-old male presented from an outside hospital after 6 days of bloody diarrhea. He was anemic, thrombocytopenic, hyponatremic, and in acute renal failure. A stool toxin assay was positive for Shiga toxin 2, confirming the diagnosis of STEC-HUS. Shortly after admission, he complained of dizziness and confusion. An MRI on day 1 revealed multiple focal infarcts, believed to be caused by HUS. Later that day, he began to seize, requiring three anti-epileptic medications and eventually intubation. Beginning on day 2 of admission, he underwent daily plasmapheresis for 3 days, with continued seizure activity and minimal improvement in neurological status. On day 5, plasmapheresis was abandoned and eculizumab 900 mg IV, in addition to vaccines and prophylactic amoxicillin was given. Within 3 hours following completion of eculizumab, the patient was awake, able to follow commands and was subsequently extubated. His neurological status continued to improve, and he transferred out of the ICU 24 hours after eculizumab. He received a second dose of eculizumab one week after the initial dose and was discharged home on a single antiepileptic medication with plans to discontinue.

Discussion: This case adds to the limited data supporting the use of eculizumab in patients with STEC-HUS. Eculizumab should be considered earlier in the disease course for patients with severe neurological symptoms of STEC-HUS. Despite the drug’s cost and potential for adverse effects, it may shorten duration of mechanical ventilation and hospitalization, likely reducing morbidity and overall costs.