皮膚色素沈着, 多彩な免疫異常を呈し, 短期間で進行した自己免疫性肝炎/原発性胆汁性肝硬変 overlap 症候群の1男性例  [in Japanese] A case of autoimmune hepatitis/primary biliary cirrhosis overlap syndrome progressing rapidly to cirrhosis with skin pigmentation and various immunological characteristics  [in Japanese]

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Abstract

症例は53歳男性.5年前に総ビリルビン3.3 mg/d<i>l</i> ,ALT 724 IU/<i>l</i> ,ALP 568 IU/<i>l</i> の肝機能異常を認めた.HBs抗原陰性,HCV抗体陰性,血清IgG正常,IgM高値,抗核抗体陰性を示した.抗ミトコンドリア抗体は20倍と陽性を示さなかったが,生化学的所見より原発性胆汁性肝硬変を疑われた.自己判断で以後は医療機関を受診していなかったが,5年後に皮膚色素沈着,腹水,黄疸を来し,紹介受診となった.5年前に正常範囲であった血清IgGは著明に高値となり,陰性であった抗核抗体が2560×以上と陽転化していた.IgMはさらに高値となり,抗ミトコンドリア抗体M2分画が93.5と強陽性であった.またLKM-1抗体,dsDNA抗体,PA-IgG,直接・間接Coombs試験も陽性であった.肝組織所見は完成した硬変像であり,血清学的所見より自己免疫性肝炎/原発性胆汁性肝硬変overlap症候群からの進展と診断した.発症から短期間での各種抗体,生化学的検査成績,臨床症状の自然経過を観察し得た貴重な自己免疫性肝炎/原発性胆汁性肝硬変overlap症候群の1男性症例であった.<br>

A 53-year-old Japanese man was found to have a liver dysfunction with a total bilirubin of 3.3 mg/d<i>l</i>, ALT 724 IU/<i>l</i>, alkaline phosphatase 568 IU/<i>l</i> with an elevation of serum IgM while serum levels of IgG, antinuclear antibody (ANA) and anti-mitochondrial antibody (AMA) showed normal. Primary biliary cirrhosis (PBC) was suspected at that time, however, he left the hospital by self-determination. Five years later, he developed liver cirrhosis with jaundice, ascites and skin pigmentation and sclerosis. Antinuclear antibody, negative 5 years before, was now positive at 1: more than 2560. Serum IgG and IgM were elevated. Anti-mitochondrial antibody M2 component, and liver-kidney microsome (LKM)-1 antibody, double strand DNA antibody, platelet-associated IgG, direct-, indirect Coombs tests were also positive. Liver histology revealed cirrhosis. He was diagnosed as having AIH/PBC overlap syndrome due to serologic features. Sequential changes of the titers of several antibodies, laboratory characteristics and clinical symptoms within short durations are discussed.<br>

Journal

  • Kanzo

    Kanzo 51(9), 513-520, 2010-09-25

    The Japan Society of Hepatology

References:  27

Codes

  • NII Article ID (NAID)
    10027469730
  • NII NACSIS-CAT ID (NCID)
    AN00047770
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    04514203
  • NDL Article ID
    10830067
  • NDL Source Classification
    ZS21(科学技術--医学--内科学)
  • NDL Call No.
    Z19-130
  • Data Source
    CJP  NDL  J-STAGE 
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