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54歳女性肺高血圧,ショック,レイノー現象,Torsade de pointes型心室性不整脈,足趾壊疽など,全身の血管炎に由来する多彩な臨床症状を呈した血管炎型のMRAの1症例を経験した.患者は抗RNP抗体が強陽性で,肺高血圧との関連が示唆された.臨床症状の安定化にはステロイドパルス療法が有用であった.
A 54 year-old woman with malignant rheumatoid arthritis was admitted with acute congestive heart failure and pulmonary hypertension. The precipitat-ing antibody to nuclear ribonucleoprotein (nRNP) was highly postive. Her hands and feet showed typical rheumatoid deformities and Raynoud's phe-nomenon. In a few days her feet became tonecrosis due to vasculitis. “Pulse” methylpredonisolone and predonisone therapy resulted in normalization of seropostivity, and left ventricular ejection fraction with symptoms of congestive heart failure, and the elimination of ECG abnormalities.
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