Hemolytic-Uremic Syndrome Medication

Updated: Oct 25, 2019
  • Author: Malvinder S Parmar, MBBS, MS, FRCPC, FACP, FASN; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Medication

Medication Summary

The U.S. Food and Drug Administration has approved two monoclonal antibodies for the treatment of hemolytic-uremic syndrome (HUS) that is not associated with Shiva-like toxin (non–Stx-HUS; atypical HUS): eculizumab and ravulizumab.

Supportive care only is used for Stx-HUS (typical HUS). 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.

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Monoclonal Antibodies

Class Summary

Agents in this category may block the formation of membrane attack complex, which can stabilize the 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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