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.

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)

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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)

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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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Media Gallery

Peripheral smear in hemolytic uremic syndrome (HUS), with findings of microangiopathic hemolytic anemia. Note schistocytes/helmet cells as well as decrease in platelets. Image courtesy of Emma Z. Du, MD.

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Author

Audrey J Tan, DO Staff Physician, Department of Emergency Medicine, State University of New York Downstate Medical Center, Kings County Hospital Center

Audrey J Tan, DO is a member of the following medical societies: American College of Emergency Physicians, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Mark A Silverberg, MD, MMB, FACEP Assistant Professor, Associate Residency Director, Department of Emergency Medicine, State University of New York Downstate College of Medicine; Consulting Staff, Department of Emergency Medicine, Staten Island University Hospital, Kings County Hospital, University Hospital, State University of New York Downstate Medical Center

Mark A Silverberg, MD, MMB, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, Council of Residency Directors in Emergency Medicine, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine, American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte Medical Center

Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Additional Contributors

William G Gossman, MD, FAAEM Associate Clinical Professor of Emergency Medicine, Creighton University School of Medicine; Chairman, Department of Emergency Medicine, Creighton University Medical Center

William G Gossman, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, William Shapiro, MD, to the development and writing of this article.