無治療の経過で腎クリーゼと心筋炎を併発した強皮症の1剖検例  [in Japanese] An autopsy case of untreated systemic sclerosis with renal crisis and myocarditis  [in Japanese]

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Author(s)

    • 小池 美菜子 KOIKE Minako
    • 東京女子医科大学八千代医療センター腎臓内科 Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 木村 和生 [他] KIMURA Kazuo
    • 東京女子医科大学八千代医療センター腎臓内科 Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 佐々木 裕子 SASAKI Yuko
    • 東京女子医科大学八千代医療センター腎臓内科 Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 高橋 正毅 TAKAHASHI Masaki
    • 東京女子医科大学八千代医療センター腎臓内科 Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 松上 桂子 MATSUGAMI Keiko
    • 東京女子医科大学八千代医療センター腎臓内科 Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 中川 典明 NAKAGAWA Noriaki
    • 東京女子医科大学八千代医療センター膠原病内科 Department of Rheumatology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 中野 雅行 NAKANO Masayuki
    • 東京女子医科大学八千代医療センター病理診断科 Department of Pathological Histology, Yachiyo Medical Center, Tokyo Women's Medical University
    • 武井 卓 TAKEI Takashi
    • 東京女子医科大学第4内科 Department of Medicine, Kidney Center, Tokyo Women's Medical University

Abstract

症例は57歳,女性.約1年前より両側手指の腫脹,両膝関節痛を自覚し,同時期より次第にADLが低下した.さらに全身の衰弱が著明となったため,2008年12月13日,家族が救急要請し,当院へ搬送された.腎不全(血清クレアチニン6.38mg/dL,尿素窒素104.9mg/dL),高カリウム血症(血清カリウム7.0mEq/L),および心電図異常を指摘され同日緊急入院となった.入院後,血液透析により,高カリウム血症は是正したが,乏尿であり,血液透析を継続した.顔貌,皮膚所見,抗Scl-70抗体陽性より,全身性強皮症と診断した.生理・画像検査上,心嚢液貯留,肺高血圧,間質性肺炎の所見を認めた.心保護目的にカプトプリル6.25mg/日を開始し,第9病日よりプレドニゾロン20mg/日の投与を開始した.徐々に血圧の変動が改善し,経口摂取も可能となっていたが,第16病日の早朝,突然心停止となり,蘇生するも循環動態は改善せず,第18病日に死亡した.病理解剖を行ったところ,皮膚硬化のほか,肺では軽度の間質性肺炎,肺高血圧性変化を認め,心臓では心筋炎・心外膜炎の所見,腎では強皮症腎クリーゼ様の所見を認めた.特に心臓ではびまん性の線維化病変と,活動性の炎症細胞浸潤の混在を認め,繰り返す心筋炎が疑われ,伝導障害から心停止に至ったと考えられた.

A 57-year-old female had demonstrated swelling of the fingers and complained of pain in both knee joints for about a year. Her ADL gradually decreased over the same period, along with the development of marked generalized weakness. The family finally transported her by ambulance to our hospital on December 13, 2008. Clinical examination demonstrated evidence of kidney failure (s-Cre 6.38mg/dL, BUN 104.9mg/dL), hyperkalemia (s-K 7.0mEq/L), and an abnormal electrocardiogram, necessitating hospitalization. After admission, hyperkalemia was corrected by hemodialysis, however, persistent oliguria necessitated maintenance hemodialysis. Systemic scleroderma was suspected from her facies and skin findings, and a definitive diagnosis was made based on a positive serological test for anti-Scl-70 antibody. Further testing demonstrated pericardial effusion, pulmonary hypertension, and interstitial pneumonia. We initiated treatment with captopril 6.25mg/day with cardioprotective intent, and prednisolone 20mg/day for the control of pericardial disease. The fluctuations in blood pressure gradually improved and the patient became able to take food. However, on the early morning of the 16th day in the hospital, the patient developed sudden cardiac arrest. While emergency cardiopulmonary resuscitation was initially successful, the patient died without regaining consciousness on the 18th day of hospitalization. At autopsy, hardening of the skin, mild interstitial pneumonia, changes in pulmonary hypertension, evidence of pericarditis and myocarditis, and also renal findings suggestive of scleroderma crisis were identified. In particular, diffuse fibrosis and inflammatory cell infiltration observed in the heart suggested that a recurrent episode of myocarditis may have caused cardiac arrest.

Journal

  • Nihon Toseki Igakkai Zasshi

    Nihon Toseki Igakkai Zasshi 43(10), 865-871, 2010-10-28

    The Japanese Society for Dialysis Therapy

References:  23

Codes

  • NII Article ID (NAID)
    10027724990
  • NII NACSIS-CAT ID (NCID)
    AN10432053
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    13403451
  • NDL Article ID
    10889183
  • NDL Source Classification
    ZS39(科学技術--医学--皮膚科学・泌尿器科学)
  • NDL Call No.
    Z19-1413
  • Data Source
    CJP  NDL  J-STAGE 
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