Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

Hirofumi Toko; Hiroto Tsuboi; Naoto Umeda; Fumika Honda; Ayako Ohyama; Hidenori Takahashi; Saori Abe; Masahiro Yokosawa; Hiromitsu Asashima; Shinya Hagiwara; Tomoya Hirota; Yuya Kondo; Isao Matsumoto; Takayuki Sumida

doi:10.2169/internalmedicine.0571-17

CASE REPORTS

Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, Hidenori Takahashi, Saori Abe, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida

Author information

Hirofumi Toko
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Hiroto Tsuboi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Naoto Umeda
Department of Rheumatology, Tsuchiura Kyodo General Hospital, Japan
Fumika Honda
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Ayako Ohyama
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Hidenori Takahashi
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Saori Abe
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Masahiro Yokosawa
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Hiromitsu Asashima
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Shinya Hagiwara
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Tomoya Hirota
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Yuya Kondo
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Isao Matsumoto
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
Takayuki Sumida
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan

Keywords: hemophagocytic syndrome, systemic lupus erythematosus, acute lupus hemophagocytic syndrome, cyclosporine A, cyclophosphamide

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 18 Pages 2747-2752

DOI https://doi.org/10.2169/internalmedicine.0571-17

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Abstract

Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond. However, the addition of cyclosporine A (CsA) led to a rapid remission from ALHS. This suggests the usefulness of CsA in the treatment of intractable, corticosteroid- and IVCY-resistant ALHS.

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