多発筋炎の診断時に原発性胆汁性肝硬変の併発を認めた1症例  [in Japanese] A case of polymyositis concomitant with primary biliary cirrhosis simultaneously  [in Japanese]

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Author(s)

    • 横須賀 淳 YOKOSUKA Jun
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 石川 智久 ISHIKAWA Tomohisa
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 中尾 裕 [他] NAKAO Yutaka
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 猿田 雅之 SARUTA Masayuki
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 穂苅 厚史 HOKARI Atsushi
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 小池 和彦 KOIKE Kazuhiko
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 北原 拓也 KITAHARA Takuya
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine
    • 原田 徹 HARADA Tohru
    • 東京慈恵会医科大学病理学講座 Department of Pathology, The Jikei University School of Medicine
    • 羽野 寛 HANO Hiroshi
    • 東京慈恵会医科大学病理学講座 Department of Pathology, The Jikei University School of Medicine
    • 田尻 久雄 TAJIRI Hisao
    • 東京慈恵会医科大学消化器肝臓内科 Department of Gastroenterology and Hepatology, The Jikei University School of Medicine

Abstract

2カ月間持続する両下肢の脱力を主訴とした症例54歳女性.肝胆道系酵素上昇に加え,両大腿の脱力と血清クレアチニンキナーゼ高値と大腿MRIで内側広筋に高信号域所見,筋生検にて単核細胞浸潤を確認し多発性筋炎と診断した.さらに,肝生検では慢性非化膿性破壊性胆管炎と小葉間胆管の破壊と細胆管増生を認め,多発性筋炎を合併した原発性胆汁性肝硬変と診断した.診断後,プレドニゾロンとウルソデオキシコール酸にて加療を開始,筋力・血液生化学検査値は共に速やかに改善した.多発性筋炎と原発性胆汁性肝硬変がほぼ同時期に発症した合併例は極めて稀であり,少数の文献報告しかない.自己免疫肝疾患に膠原病を合併した症例として貴重と考え,若干の文献的考察を加え報告した.<br>

A 54-year-old woman experienced continuous proximal muscle weakness in both legs 2 months before. Her laboratory findings showed remarkably high levels of serum creatine kinase, gamma-glutamyl transferase, and alkaline phosphatase. Magnetic resonance imaging (MRI) showed a high-intensity signal area at the vastus medialis muscle. Muscle biopsy findings revealed mononuclear inflammatory cell infiltration in endomysial connective tissue. On ultrasonography, we observed fibrosis change in the liver parenchyma. Histological examination of the liver revealed chronic nonsuppurative destructive cholangitis. We diagnosed the patient with polymyositis concurrent with primary biliary cirrhosis. Immediately after the patient was administered prednisolone and ursodeoxycholic acid, her muscle weakness and clinical data improved. Only a few previous studies have reported cases of polymyositis concurrent with primary biliary cirrhosis. Therefore, we believe that our study is unique and particularly useful for further study of autoimmune liver diseases.<br>

Journal

  • Kanzo

    Kanzo 53(4), 216-224, 2012-04-25

    The Japan Society of Hepatology

References:  38

Codes

  • NII Article ID (NAID)
    10030625388
  • NII NACSIS-CAT ID (NCID)
    AN00047770
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    04514203
  • NDL Article ID
    023602394
  • NDL Call No.
    Z19-130
  • Data Source
    CJP  NDL  J-STAGE 
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