DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions

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  • Sugie Kazuma
    Department of Neurology, Nara Medical University School of Medicine, Japan
  • Tonomura Yasuyo
    Department of Neurology, Nara Medical University School of Medicine, Japan
  • Ueno Satoshi
    Department of Neurology, Nara Medical University School of Medicine, Japan

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タイトル別名
  • Characterization of Dermatomyositis with Coexistence of Anti-Jo-1 and Anti-SRP Antibodies

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We describe a patient with dermatomyositis who presented with rapidly developing severe muscle weakness complicated by massive pleural effusion with interstitial lung disease. Myopathological analysis was suggestive of dermatomyositis. This patient showed both anti-Jo-1 and anti-SRP antibodies in serum. To our knowledge, the coexistence of these two myositis-specific autoantibodies (MSA) is considered extremely rare and is clearly an exception to the rule of having only one MSA. Our findings provide compelling evidence that the coexistence of these two MSAs may lead to more severe clinical symptoms, interacting in a complex fashion, thus expanding the clinical spectrum of idiopathic inflammatory myopathy.<br>

収録刊行物

  • Internal Medicine

    Internal Medicine 51 (7), 799-802, 2012

    一般社団法人 日本内科学会

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