心膜液を伴う強皮症腎クリーゼの1例  [in Japanese] Scleroderma renal crisis with pericardial effusion  [in Japanese]

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Author(s)

    • 大瀬 貴元 OHSE Takamoto
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo
    • 上田 浩平 UEDA Kohei
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo
    • 正路 久美 SHOJI Kumi
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo
    • 藤乗 嗣泰 TOJO Akihiro
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo
    • 関 常司 SEKI George
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo
    • 藤田 敏郎 FUJITA Toshiro
    • 東京大学医学部附属病院腎臓・内分泌内科 Division of Nephrology and Endocrinology, University of Tokyo

Abstract

症例は62歳,女性.生来健康だったが3年前から両手指ソーセージ様腫脹,指尖潰瘍とRaynaud症状が出現,口周囲皺の減少もみられ,1年前から労作時呼吸困難も出現した.近医で漢方薬を処方されたが症状に改善はみられなかった.起座呼吸を主訴に前医に入院,乳頭浮腫を伴う高血圧,大量心膜液,うっ血性心不全,肺線維症,破砕赤血球像と腎機能障害を認めた.入院後は降圧治療を行い血圧は150/80 mmHg程度まで改善,破砕赤血球も消失したが,腎機能悪化と浮腫の増悪を認め入院3日後に当科転院となった.入院時は上記皮膚所見に加え収縮期心雑音と肺野の乾性ラ音,四肢の浮腫を認め,尿蛋白・尿潜血を伴う腎機能障害,胸部X線で心拡大と肺うっ血像を認めた.心エコーでは心膜液貯留があり胸部CTでは両側肺野に軽度の間質性変化も認めた.抗Scl-70抗体が陽性であり全身性強皮症に伴う強皮症腎クリーゼと診断,心膜液を伴ううっ血性心不全を併発したと考えた.ACE(angiotensin-converting enzyme)阻害薬とCa拮抗薬で降圧を行うとともに血液透析を開始,透析前血圧は138/68 mmHg程度まで改善し,肺うっ血と四肢の浮腫は消失したため入院27日目に退院となった.本邦での全身性強皮症患者における腎クリーゼの発生頻度は海外にくらべ少ないといわれており,今回心膜液を伴い血液透析導入に至った重症例を経験したので報告する.

A 62-year-old woman with no contributory medical history transferred to our hospital. Three years before presentation, she had complained of sausage-like fingers, Raynaud's phenomenon, digital-tip ulcers and decreased wrinkles around her mouth. One year before presentation, she had dyspnea on effort. Though Chinese herbal medicine was administered, these symptoms did not improve. Three days before presentation, she had been admitted to a local hospital because of orthopnea. On admission, hypertension with papilledema, massive pericardial effusion, congestive heart failure, pulmonary fibrosis, red cell fragmentation on peripheral blood smear and renal impairment were present. On anti-hypertensive therapy, blood pressure decreased to 150/80 mmHg and red cell fragmentation on peripheral blood smear disappeared. However, renal dysfunction and edema of extremities developed and she transferred to our hospital. On admission, skin scleroderma, skin pigmentation, systolic murmur, fine crackles and edema of extremities were present. Urinalysis showed both proteinuria and microhematuria and chest X ray showed cardiomegaly and lung congestion. Echocardiography demonstrated massive pericardial effusion and chest computed tomography scan showed mild interstitial change of the bilateral lower lung fields. Taking positive anti-Scl-70 antibody into consideration, she was diagnosed with scleroderma renal crisis and congestive heart failure with pericardial effusion. Both ACE inhibitor and hemodialysis were started and blood pressure before one session of hemodialysis decreased to 138/68 mmHg. Since congestion and edema of extremities improved, she was discharged from hospital on the 27<SUP>th</SUP> hospital day. The frequency of scleroderma renal crisis is lower in Japan than in western countries. We report a case of severe scleroderma renal crisis with pericardial effusion that progressed to end stage renal disease.

Journal

  • Nihon Toseki Igakkai Zasshi

    Nihon Toseki Igakkai Zasshi 44(5), 455-461, 2011-05-28

    The Japanese Society for Dialysis Therapy

References:  29

Codes

  • NII Article ID (NAID)
    10029406882
  • NII NACSIS-CAT ID (NCID)
    AN10432053
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    13403451
  • NDL Article ID
    11133518
  • 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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