An autopsy case of untreated systemic sclerosis with renal crisis and myocarditis

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  • Kawanishi Kunio
    Department of Medicine, Kidney Center, Tokyo Women's Medical University
  • Koike Minako
    Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Kimura Kazuo
    Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Sasaki Yuko
    Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Takahashi Masaki
    Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Matsugami Keiko
    Department of Nephrology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Nakagawa Noriaki
    Department of Rheumatology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Nakano Masayuki
    Department of Pathological Histology, Yachiyo Medical Center, Tokyo Women's Medical University
  • Takei Takashi
    Department of Medicine, Kidney Center, Tokyo Women's Medical University
  • Nitta Kousaku
    Department of Medicine, Kidney Center, Tokyo Women's Medical University

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Other Title
  • 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
  • 症例報告 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
  • ショウレイ ホウコク ムチリョウ ノ ケイカ デ ジンクリーゼ ト シンキンエン オ ヘイハツ シタ キョウヒショウ ノ 1 ボウケンレイ

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Abstract

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.

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