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

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.

• Authors: 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
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 57 Issue 23 Pg. 3473-3478 (Dec 01 2018) ISSN: 1349-7235 [Electronic] Japan
• PMID: 29984753 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Autoantibodies
• Immunosuppressive Agents
• Cyclophosphamide
• IFIH1 protein, human
• Interferon-Induced Helicase, IFIH1
• Tacrolimus
• Methylprednisolone

Topics

• Adrenal Cortex Hormones (adverse effects, therapeutic use)
• Autoantibodies (blood)
• Combined Modality Therapy
• Cyclophosphamide (administration & dosage, therapeutic use)
• Dermatomyositis (complications, immunology, therapy)
• Disease Progression
• Female
• Humans
• Immunosuppressive Agents (administration & dosage, adverse effects, therapeutic use)
• Interferon-Induced Helicase, IFIH1 (immunology)
• Lung Diseases, Interstitial (complications)
• Lymphohistiocytosis, Hemophagocytic (complications, immunology, therapy)
• Methylprednisolone (adverse effects, therapeutic use)
• Middle Aged
• Plasmapheresis
• Pulse Therapy, Drug
• Tacrolimus (adverse effects, therapeutic use)