title: Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab.
abstract: Thrombotic microangiopathy (TMA) is a severe disorder with poor outcomes. The cause is unknown for many patients, although TMA is associated with connective tissue disorders, including systemic lupus erythematosus (SLE). While uncommon, TMA is one of the most serious complications of SLE and in many cases may be resistant to therapy. We report a patient with SLE complicated by TMA that was refractory to standard therapy but responded well to eculizumab, with continued remission after 1 year of follow-up. Eculizumab might be useful in the management of resistant cases of TMA caused by SLE.
authors.affiliation: Division of Nephrology, University of Kentucky, Lexington, KY; Mansoura University, Egypt. Electronic address: amr.elhusseini.moh@uky.edu.
authors.forename: Amr
authors.initials: A
authors.lastname: El-Husseini
authors.affiliation: Division of Nephrology, University of Kentucky, Lexington, KY.
authors.forename: Schot
authors.initials: S
authors.lastname: Hannan
authors.affiliation: Division of Nephrology, University of Kentucky, Lexington, KY.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Awad
authors.affiliation: Division of Nephrology, University of Kentucky, Lexington, KY.
authors.forename: Stuart
authors.initials: S
authors.lastname: Jennings
authors.affiliation: Department of Pathology, University of Kentucky, Lexington, KY.
authors.forename: Virgilius
authors.initials: V
authors.lastname: Cornea
authors.affiliation: Division of Nephrology, University of Kentucky, Lexington, KY.
authors.forename: B Peter
authors.initials: BP
authors.lastname: Sawaya
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage / Q000009:adverse effects*; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D001706:Biopsy / Q000379:methods; D016903:Drug Monitoring / Q000379:methods; D005260:Female; D005919:Glomerular Filtration Rate / Q000187:drug effects; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007668:Kidney / Q000473:pathology / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D008181:Lupus Nephritis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D012074:Remission Induction; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007155:Immunologic Factors; C481642:eculizumab
keywords: Thrombotic microangiopathy (TMA); acute kidney injury (AKI); atypical hemolytic uremic syndrome (aHUS); eculizumab; kidney biopsy; lupus nephritis; proteinuria; systemic lupus erythematosus (SLE)