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

• Fujita Yuya

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Fukui Shoichi

  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

• Suzuki Takahisa

  Department of Rheumatology, Japanese Red Cross Nagasaki Genbaku Hospital, Japan

• Ishida Midori

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Endo Yushiro

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Tsuji Sousuke

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Takatani Ayuko

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Igawa Takashi

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Shimizu Toshimasa

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Umeda Masataka

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan Medical Education Development Center, Nagasaki University Hospital, Japan

• Sumiyoshi Remi

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Nishino Ayako

  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

• Koga Tomohiro

  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

• Kawashiri Shin-ya

  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

• Iwamoto Naoki

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Ichinose Kunihiro

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Tamai Mami

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Nakamura Hideki

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

• Origuchi Tomoki

  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

• Abe Kuniko

  Department of Pathology, Nagasaki University Hospital, Japan

• Kawakami Atsushi

  Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

抄録

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.

収録刊行物

• Internal Medicine

  Internal Medicine 57 (23), 3473-3478, 2018-12-01

  一般社団法人 日本内科学会

キーワード

• anti-MDA5 antibody-positive dermatomyositis
• autoimmune disease
• hemophagocytic syndrome
• corticosteroid therapy
• cyclophosphamide
• plasmapheresis

詳細情報

• CRID: 1390001288095362688
• NII論文ID: 130007539684
• DOI: 10.2169/internalmedicine.1121-18
• ISSN: 13497235
• ISSN: 09182918
• HANDLE: 10069/38763
• PubMed: 29984753
• Web Site: https://nagasaki-u.repo.nii.ac.jp/records/1498
• Web Site: https://www.jstage.jst.go.jp/article/internalmedicine/57/23/57_1121-18/_pdf
• 本文言語コード: en
• データソース種別:
  • JaLC
  • IRDB
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• 抄録ライセンスフラグ: 使用不可