Hemolytic Uremic Syndrome in Emergency Medicine Medication

Updated: Jun 24, 2021
  • Author: Audrey J Tan, DO; Chief Editor: Steven C Dronen, MD, FAAEM  more...
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Medication

Medication Summary

For hemolytic uremic syndrome (HUS) related to Shiga toxin (ie, typical HUS), supportive care only is used. Medications for supportive care may include angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-receptor blockers (ARBs) for control of hypertension, or phenytoin for prevention of seizures. For complement-mediated HUS (ie, atypical HUS), two monoclonal antibodies that block complement component C5 have been approved for use: eculizumab and ravulizumab.

Refractory cases have been treated with vincristine or cyclosporine. Steroids are of questionable benefit, as are antiplatelet agents such as aspirin or dipyridamole. Fibrinolytic therapy is not only ineffective but it also increases the risk of bleeding. 

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Complement Inhibitors

Class Summary

Two monoclonal antibodies that block complement C5 are approved for use in atypical hemolytic uremic syndrome (HUS).  These agents may block the formation of membrane attack complex, which can stabilize hemoglobin and reduce the need for RBC transfusions.

Eculizumab (Soliris)

Monoclonal blocking antibody to complement protein C5; inhibits cleavage to C5a and C5b, thus preventing terminal complement complex C5b-9, thereby preventing RBC hemolysis. Inhibits terminal complement mediated intravascular hemolysis in PNH patients and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS.

Ravulizumab (Ravulizumab-cwvz, Ultomiris)

Ravulizumab is a monoclonal blocking antibody to complement protein C5; it inhibits cleavage to C5a and C5b, thus preventing terminal complement complex C5b-9, thereby preventing RBC hemolysis. It inhibits terminal complement-mediated intravascular hemolysis in patients with paroxysmal nocturnal hemoglobinuria and complement-mediated thrombotic microangiopathy in patients with aHUS.

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