Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Anti-MDA5 Antibody-positive Dermatomyositis Complicated by Autoimmune-associated Hemophagocytic Syndrome That Was Successfully Treated with Immunosuppressive Therapy and Plasmapheresis
Yuya FujitaShoichi FukuiTakahisa SuzukiMidori IshidaYushiro EndoSousuke TsujiAyuko TakataniTakashi IgawaToshimasa ShimizuMasataka UmedaRemi SumiyoshiAyako NishinoTomohiro KogaShin-ya KawashiriNaoki IwamotoKunihiro IchinoseMami TamaiHideki NakamuraTomoki OriguchiKuniko AbeAtsushi Kawakami
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JOURNALS OPEN ACCESS

2018 Volume 57 Issue 23 Pages 3473-3478

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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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© 2018 by The Japanese Society of Internal Medicine
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