ABSTRACT
Although rare, atypical hemolytic syndrome (aHUS) has been recognized as one of the direst complications of systemic lupus erythematosus (SLE). Furthermore, the diagnosis of coexisting aHUS and SLE is a diagnostic dilemma with similar clinical characteristics between both entities. Eculizumab is an effective treatment for complement-mediated atypical hemolytic uremic syndrome, but much is still to be learned about optimal treatment duration and if eculizumab can be discontinued without thrombotic microangiopathy reoccurrence. Here, we report a pediatric case of severe SLE complicated by aHUS that responded favorably to eculizumab, followed by successful discontinuation without recurrence of aHUS despite having numerous identified risk factors.
