Anti-MDA5 Antibody-positive Dermatomyositis Complicated by Autoimmune-associated Hemophagocytic Syndrome That Was Successfully Treated with Immunosuppressive Therapy and Plasmapheresis

Yuya Fujita; Shoichi Fukui; Takahisa Suzuki; Midori Ishida; Yushiro Endo; Sousuke Tsuji; Ayuko Takatani; Takashi Igawa; Toshimasa Shimizu; Masataka Umeda; Remi Sumiyoshi; Ayako Nishino; Tomohiro Koga; Shin-ya Kawashiri; Naoki Iwamoto; Kunihiro Ichinose; Mami Tamai; Hideki Nakamura; Tomoki Origuchi; Kuniko Abe; Atsushi Kawakami

doi:10.2169/internalmedicine.1121-18

CASE REPORTS

Anti-MDA5 Antibody-positive Dermatomyositis Complicated by Autoimmune-associated Hemophagocytic Syndrome That Was Successfully Treated with Immunosuppressive Therapy and Plasmapheresis

Yuya Fujita, Shoichi Fukui, Takahisa Suzuki, Midori Ishida, Yushiro Endo, Sousuke Tsuji, Ayuko Takatani, Takashi Igawa, Toshimasa Shimizu, Masataka Umeda, Remi Sumiyoshi, Ayako Nishino, Tomohiro Koga, Shin-ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Kuniko Abe, Atsushi Kawakami

Author information

Yuya Fujita
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Shoichi Fukui
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takahisa Suzuki
Department of Rheumatology, Japanese Red Cross Nagasaki Genbaku Hospital, Japan
Midori Ishida
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Yushiro Endo
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Sousuke Tsuji
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Ayuko Takatani
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takashi Igawa
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Toshimasa Shimizu
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Masataka Umeda
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Medical Education Development Center, Nagasaki University Hospital, Japan
Remi Sumiyoshi
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Ayako Nishino
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Center for Comprehensive Community Care Education Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Tomohiro Koga
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Shin-ya Kawashiri
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Naoki Iwamoto
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Kunihiro Ichinose
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Mami Tamai
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hideki Nakamura
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Tomoki Origuchi
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Rehabilitation Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan
Kuniko Abe
Department of Pathology, Nagasaki University Hospital, Japan
Atsushi Kawakami
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

Keywords: anti-MDA5 antibody-positive dermatomyositis, autoimmune disease, hemophagocytic syndrome, corticosteroid therapy, cyclophosphamide, plasmapheresis

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 23 Pages 3473-3478

DOI https://doi.org/10.2169/internalmedicine.1121-18

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Abstract

A 56-year-old Japanese woman with muscle weakness, increased creatine kinase and aldolase levels, and characteristic cutaneous lesions was diagnosed with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis. She also had interstitial lung disease (ILD). After corticosteroid and tacrolimus combination therapy was started, bicytopenia and elevated serum ferritin and transaminase emerged. Because the bone marrow tissues were hypoplastic with hemophagocytes, she was diagnosed with concomitant autoimmune-associated hemophagocytic syndrome (HPS). Intravenous cyclophosphamide pulse therapy and plasmapheresis were performed. The laboratory findings indicated improved abnormalities, and the ILD did not progress. Anti-MDA5 antibody-positive dermatomyositis can be complicated by HPS.

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