Atypical haemolytic uraemic syndrome

Introduction Atypical haemolytic uremic syndrome is a result of a spectrum of diseases. Disorders of complement regulation are the most important reasons in the aetiology. It is associated with defective regulation of the alternative complement pathway in over 50% of cases. Clinical abnormalities are related with the presence of thrombotic microangiopathy. Patients with atypical haemolytic 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. We discuss haemolytic uremic syndrome and treatment options. Conclusion Related to increase of experiences, eculizumab therapy may be the firstline treatment. We do not know optimal duration of eculizumab therapy. We do not know also in which patient a severe relapse could be developed. At this moment we can suggest that in both cases eculizumab is life-saving and enhancing the