A Case Of Atypical Hemolytic Uremic Syndrome With Positive Anti-Factor H Autoantibody

Atypical hemolytic uremic syndrome (aHUS) is defined as HUS caused by dysregulation of the complement alternative pathway. Although the incidence rate of aHUS is less than 1/100,000, the prognosis is very poor. The main pathogenesis of aHUS is primarily the over-activation of the complement alternative pathway induced by the abnormalities of the Factor H protein family. aHUS associated with anti-Factor H autoantibodies accounted for approximately 10% of all aHUS cases. Herein is reported an adult with aHUS who exhibited positive anti-Factor H autoantibodies and homozygous deletion for complement Factor H-related protein 1 (CFHR1).