Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab

Amr El-Husseini; Schot Hannan; Ahmed Awad; Stuart Jennings; Virgilius Cornea; B Peter Sawaya

doi:10.1053/j.ajkd.2014.07.031

Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab

Am J Kidney Dis. 2015 Jan;65(1):127-30. doi: 10.1053/j.ajkd.2014.07.031. Epub 2014 Nov 15.

Authors

Amr El-Husseini¹, Schot Hannan², Ahmed Awad², Stuart Jennings², Virgilius Cornea³, B Peter Sawaya²

Affiliations

¹ Division of Nephrology, University of Kentucky, Lexington, KY; Mansoura University, Egypt. Electronic address: [email protected].
² Division of Nephrology, University of Kentucky, Lexington, KY.
³ Department of Pathology, University of Kentucky, Lexington, KY.

PMID: 25446020
DOI: 10.1053/j.ajkd.2014.07.031

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.

Keywords: Thrombotic microangiopathy (TMA); acute kidney injury (AKI); atypical hemolytic uremic syndrome (aHUS); eculizumab; kidney biopsy; lupus nephritis; proteinuria; systemic lupus erythematosus (SLE).

Publication types

Case Reports

MeSH terms

Acute Kidney Injury* / diagnosis
Acute Kidney Injury* / drug therapy
Acute Kidney Injury* / etiology
Acute Kidney Injury* / physiopathology
Antibodies, Monoclonal, Humanized / administration & dosage
Antibodies, Monoclonal, Humanized / adverse effects*
Atypical Hemolytic Uremic Syndrome* / drug therapy
Atypical Hemolytic Uremic Syndrome* / etiology
Atypical Hemolytic Uremic Syndrome* / physiopathology
Biopsy / methods
Drug Monitoring / methods
Female
Glomerular Filtration Rate / drug effects
Humans
Immunologic Factors / administration & dosage
Kidney* / pathology
Kidney* / physiopathology
Lupus Erythematosus, Systemic / complications*
Lupus Erythematosus, Systemic / diagnosis
Lupus Nephritis / complications
Lupus Nephritis / diagnosis
Lupus Nephritis / physiopathology
Remission Induction
Thrombotic Microangiopathies* / diagnosis
Thrombotic Microangiopathies* / drug therapy
Thrombotic Microangiopathies* / etiology
Thrombotic Microangiopathies* / physiopathology
Treatment Outcome
Young Adult

Substances

Antibodies, Monoclonal, Humanized
Immunologic Factors
eculizumab