A case of renal recovery in atypical hemolytic uremic syndrome treated with eculizumab

Abstract

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare, life-threatening disease that can occur at any age and be sporadic or familial. aHUS is caused by an uncontrolled activation of the complement system. Plasma Exchange (PE) has been the standard treatment for years with poor results, leading approximately 40% of patients to end-stage renal disease (ESRD) or death during the first clinical manifestation. Eculizumab, an humanized monoclonal antibody directed against complement component C5, has emerged in the last few years as a new therapheutic aproach with promising results. Our goal is to present a case of an adult patient where eculizumab was sucessfully used as upfront therapy avoiding the potential significant morbidity of PE.

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment

“…Although eculizumab is proven to be effective as an initial treatment for aHUS because it is a complement inhibitor, it increases lifethreatening infections by encapsulated micro organisms, such as Neisseria meningitidis, Streptococcus pneumoniae, and Haemophilus influenzae 3,5,8,9) . Therefore, patients treated with eculizumab should be vaccinated against these organisms at least 2 weeks prior to initiating treatment.…”

“…Therefore, patients treated with eculizumab should be vaccinated against these organisms at least 2 weeks prior to initiating treatment. If the patient could not be vaccinated before eculizumab treatment, appropriate antibiotics should be administered for 2 weeks for prophylaxis against bacterial meningitis 5,7) .…”