Complement inhibition therapy in patients with atypical hemolytic uremic syndrome and secondary microangiopathies

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by uncontrolled complement activation and leading to thrombocytopenia, microangiopathic hemolysis acute kidney injury. Eculizumab, humanized monoclonal antibody that inhibits the cleavage protein C5, effectively reverses laboratory evidence TMA improves function in most patients with aHUS. Hereditary aHUS should be differentiated from secondary HUS associated drugs, autoimmune diseases (e.g. antiphospholipid syndrome), malignancies, infections, solid organ transplantation. Case reports retrospective studies suggest eculizumab may effective proportion TMA, although its efficacy regimens administration evaluated additional studies.