What are the treatments available for atypical HUS?
Anticomplement therapy is now the standard of care for the treatment of aHUS. Eculizumab is a humanized monoclonal antibody directed against C5 of the complement cascade and thereby inhibits the formation of C5a and C5b-9 membrane attack complex. Its use may be limited in patients with C5 mutations. In patients with autoantibodies to CFH, eculizumab can be used in addition to the consideration of immunosuppression with corticosteroids. The ongoing considerations with regards to eculizumab use are the high cost of the medication and no clear criteria of when to stop the medication. Plasmapheresis remains a part of initial therapy because there may be a delay in diagnosis and/or obtaining the drug.
