title: Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus.
abstract: Atypical hemolytic uremic syndrome is characterized by the presence of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury. In this report, we describe two patients with systemic lupus erythematosus who presented with findings compatible with atypical hemolytic uremic syndrome, complicated by acute kidney injury that was refractory to conventional therapies. Both patients exhibited a response to eculizumab, a monoclonal antibody to complement protein C5, with stabilization of their platelet count. On 1-year follow-up from their initial presentation, their hematologic disease remained in remission without recurrence.
authors.affiliation: From the *Pritzker School of Medicine, †Section of Rheumatology and Gwen Knapp Center for Lupus and Immunology Research, and ‡Section of Hematology and Oncology, University of Chicago, Chicago, IL.
authors.forename: Rene S
authors.initials: RS
authors.lastname: Bermea
authors.forename: Niharika
authors.initials: N
authors.lastname: Sharma
authors.forename: Kenneth
authors.initials: K
authors.lastname: Cohen
authors.forename: Vladimir M
authors.initials: VM
authors.lastname: Liarski
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D010976:Platelet Count / Q000379:methods; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007155:Immunologic Factors; C481642:eculizumab