Table of Contents Author Guidelines Submit a Manuscript
Autoimmune Diseases
Volume 2011, Article ID 483642, 6 pages
Case Report

A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

1Department of Rheumatology, School of Medicine, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
2Department of Internal Medicine and Rheumatology, Juntendo University Urayasu Hospital, Chiba 279-0021, Japan
3Department of Internal Medicine, Juntendo University Urayasu Hospital, Chiba 279-0021, Japan
4Institute for Environment and Gender Specific Medicine, Juntendo University Graduate School of Medicine, Chiba 279-0021, Japan

Received 16 March 2011; Accepted 16 May 2011

Academic Editor: R. Cervera

Copyright © 2011 Risa Yamada et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.