Successful Discontinuation of Eculizumab in a Pediatric Patient With Atypical Hemolytic Uremic Syndrome and Underlying Systematic Lupus Erythematosus.

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.