Thrombotic Microangiopathy in Systemic Lupus Erythematosus: Efficacy of Eculizumab

• Published in: American journal of kidney diseases Vol. 65; no. 1; pp. 127 - 130
• Main Authors: El-Husseini, Amr, MD, Hannan, Schot, MD, Awad, Ahmed, MD, Jennings, Stuart, MD, Cornea, Virgilius, MD, Sawaya, B. Peter, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.01.2015
• Subjects: acute kidney injury (AKI); 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 (aHUS); Atypical Hemolytic Uremic Syndrome - drug therapy; Atypical Hemolytic Uremic Syndrome - etiology; Atypical Hemolytic Uremic Syndrome - physiopathology; Biopsy - methods; Drug Monitoring - methods; eculizumab; Female; Glomerular Filtration Rate - drug effects; Humans; Immunologic Factors - administration & dosage; Kidney - pathology; Kidney - physiopathology; kidney biopsy; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; lupus nephritis; Lupus Nephritis - complications; Lupus Nephritis - diagnosis; Lupus Nephritis - physiopathology; Nephrology; proteinuria; Remission Induction; systemic lupus erythematosus (SLE); Thrombotic Microangiopathies - diagnosis; Thrombotic Microangiopathies - drug therapy; Thrombotic Microangiopathies - etiology; Thrombotic Microangiopathies - physiopathology; Thrombotic microangiopathy (TMA); Treatment Outcome; Young Adult; acute kidney injury (AKI); atypical hemolytic uremic syndrome (aHUS); Thrombotic microangiopathy (TMA); systemic lupus erythematosus (SLE); kidney biopsy; proteinuria; lupus nephritis; eculizumab
• ISSN: 0272-6386; 1523-6838
• DOI: 10.1053/j.ajkd.2014.07.031

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.