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Case Reports in Rheumatology
Volume 2017, Article ID 4159727, 3 pages
Case Report

Successful Treatment of Hemophagocytic Lymphohistiocytosis Associated with Lupus Nephritis by Using Mycophenolate Mofetil

1Department of Medicine and Clinical Science, Division of Cardiology and Clinical Immunology, Yamaguchi University Graduate School of Medicine, Ube, Japan
2Division of Endocrinology, Metabolism, Hematological Science and Therapeutics, Yamaguchi University Graduate School of Medicine, Ube, Japan

Correspondence should be addressed to Makoto Kubo;

Received 27 May 2017; Accepted 12 July 2017; Published 14 September 2017

Academic Editor: Shoichiro Ikuyama

Copyright © 2017 Takashi Nawata 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.


An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who developed HLH associated with lupus nephritis. Initially, her HLH was refractory to treatment with a corticosteroid, tacrolimus, and mizoribine. However, alternative treatment with a corticosteroid, mycophenolate mofetil, and tacrolimus improved both her HLH and lupus nephritis. This case suggests the possibility of mycophenolate mofetil as a key drug for treating HLH associated with SLE.