Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Nephrol. Nov 6, 2015; 4(5): 528-531
Published online Nov 6, 2015. doi: 10.5527/wjn.v4.i5.528
Lupus-associated thrombotic thrombocytopenic purpura-like microangiopathy
Daniel Blum, Geoffrey Blake
Daniel Blum, Geoffrey Blake, Department of Hematology, McGill University Health Center, Montreal, Quebec H3G1A4, Canada
Daniel Blum, Department of Internal Medicine (G-050), Jewish General Hospital, Montreal, Quebec H3T1E2, Canada
Author contributions: Blum D performed the chart review of the case being described, searched, reviewed and critically appraised the literature for cases of thrombotic microangiopathy in patients with lupus; Blake G was involved in making the presumptive diagnosis of the TTP-like syndrome in the patient with lupus described in this case report, and in initiating the management of this rare complication of lupus; Blake G also reviewed and amended this case report manuscript prior to its submission.
Institutional review board statement: Not applicable; this report describes the clinical course of a patient under the care of the general medicine team at the Montreal General Hospital.
Informed consent statement: Explicit written informed consent was obtained from the patient described in this case prior to submitting this case report. A copy of the consent form signed by this patient can be provided upon request.
Conflict-of-interest statement: The authors have no conflicts of interest to report.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Daniel Blum, MDCM, Department of Internal Medicine (G-050), Jewish General Hospital, 3755 Cote Saint Catherine, Montreal, Quebec H3T1E2, Canada. daniel.blum@mail.mcgill.ca
Telephone: +1-514-7582586
Received: November 19, 2014
Peer-review started: November 22, 2014
First decision: December 12, 2014
Revised: September 11, 2015
Accepted: September 29, 2015
Article in press: September 30, 2015
Published online: November 6, 2015
Processing time: 358 Days and 1 Hours
Core Tip

Core tip: In patients with lupus who develop thrombotic microangiopathy, early initiation of plasma exchange appears to carry a survival benefit - even in those patients whose ADAMTS13 activity levels are not severely deficient. This improved survival has become apparent in the last ten years during which time seven cases of thrombotic microangiopathy complicating lupus and treated with plasma exchange have been reported. The present article describes the eighth such case, reviews the previously described cases and outcomes of microangiopathy in lupus, and hypothesizes as to why plasma exchange appears to be beneficial in this subset of patients with atypical haemolytic uremic syndrome.