Abstract:

Atypical hemolytic uremic syndrome is the result of a spectrum of diseases. Disorders of complement regulation are the most important reasons in the etiology. It is associated with defective regulation of the alternative complement pathway in over 50% of the cases. Clinical abnormalities are related with the presence of thrombotic microangiopathy. Patients with atypical hemolytic uremic syndrome have a poor prognosis with a high mortality and morbidity in the acute phase of the disease and progression to end-stage renal disease in 50% of the cases. Various extra renal complications due to systemic thrombotic microangiopathy may occur in HUS, including neurological, pancreatic and cardiac involvement. Eculizumab is a humanized monoclonal anti-C5 antibody. It blocks the alternative complement pathway at the level of proinflammatory C5a and lytic C5b-9 complex generation. With the increase in experience, eculizumab therapy may be the first-line treatment. We do not know the optimal duration of eculizumab therapy. We also do not know in which patient a severe relapse could develop. At this moment we can suggest that eculizumab is life-saving and enhances the quality of life

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