A case of anti-aminoacyl tRNA synthetase antibody syndrome complicated by hemophagocytic syndrome

  • AZUMA Kota
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • TAMURA Masao
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • KURAJOH Masafumi
    Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Hyogo College of Medicine
  • HOSONO Yuji
    Department of Rheumatology and Clinical Immunology, Kyoto University Graduate school of Medicine
  • NAKAJIMA Ran
    Department of Rheumatology and Clinical Immunology, Kyoto University Hospital
  • TSUBOI Kazuyuki
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • ABE Takeo
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • OGITA Chie
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • YOKOYAMA Yuichi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • FURUKAWA Tetsuya
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • YOSHIKAWA Takahiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • SAITO Atsushi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • NISHIOKA Aki
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • SEKIGUCHI Masahiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • AZUMA Naoto
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • KITANO Masayasu
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • TSUNODA Shinichiro
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • OMURA Koichiro
    Department of Rheumatology and Clinical Immunology, Kyoto University Graduate school of Medicine
  • KOYAMA Hidenori
    Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Hyogo College of Medicine
  • MATSUI Kiyoshi
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine
  • MIMORI Tsuneyo
    Department of Rheumatology and Clinical Immunology, Kyoto University Graduate school of Medicine
  • SANO Hajime
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine

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Other Title
  • 抗ARS抗体症候群に血球貪食症候群(HPS)が合併した1例

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Abstract

<p>  A 48-year-old woman had suffered from a fever and general fatigue, and visited the other hospital for fever elevation in November 2013, at which time interstitial lung disease was revealed. In January 2014, she experienced an eruption in the hand and developed peripheral blood flow damage. Under a diagnosis of adult Still's disease, the patient was administered 0.5 mg of betamethasone as well as cyclosporin at 75 mg/day. In November 2014, general fatigue, fever, and headache were noted, while MRI revealed an enlarged hypophysis and laboratory findings were positive for the anti-pituitary cell antibody, thus a diagnosis of autoimmune hypophysitis was made. Although disease activity was low, she requested hospitalization and was admitted by the Division of Endocrinology and Metabolism at our hospital in May 2015, though only observed. Fever developed again, along with interstitial lung disease, Raynaud's phenomenon, and pain in the crural area again, and we considered the possibility of another disease. After stopping administration of betamethasone and cyclosporin, we made a diagnosis of anti-aminoacyl tRNA synthetase antibody syndrome, and administered methylprednisolone at 500 mg for 3 days as well as prednisolone at 35 mg/day following steroid pulse therapy. Although her condition soon improved, fever, muscle pain, and pancytopenia returned after 3 days. Bone marrow findings revealed the existence of hemophagocytosis, for which we again gave methylprednisolone at 500 mg for 3 days and cyclosporin at 125 mg/day. Thereafter, the patient recovered and was discharged from the hospital.</p>

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