Maekawa K, Fujimoto T, Uemura S, Kanauchi M, Dohi K
First Department of Internal Medicine, Nara Medical University.
Nihon Rinsho Meneki Gakkai Kaishi. 1997 Feb;20(1):79-85. doi: 10.2177/jsci.20.79.
This report described a 44 years-old female mixed connective tissue disease (MCTD) patient presenting pulmonary hypertension, successfully treated with angiotensin I converting enzyme (ACE) inhibitor. The patient was diagnosed as having MCTD because of Raynaud's phenomenon, swollen hand, and elevated level of anti-U 1 RNP antibodies. She was admitted to our hospital one year after the diagnosis of MCTD, because she experienced dyspnea and pretibial edema. A diagnosis of pulmonary hypertension was made by echocardiography and catheterization study. Pulmonary artery pressure and pulmonary artery resistance declined and returned gradually to normal during a period of three months with prednisolone and enalapril (10 mg/day) therapy. Pulmonary hypertension is one of fetal complications with MCTD, and 5 year mortality rate is reported less than 50%. However, a successful therapy for the pulmonary hypertension with MCTD has not been established yet. We considered that ACE inhibitor might be useful to control the pulmonary hypertension with MCTD and could improve the prognosis of MCTD.
本报告描述了一名44岁的混合性结缔组织病（MCTD）女性患者，该患者出现肺动脉高压，经血管紧张素I转换酶（ACE）抑制剂治疗成功。该患者因雷诺现象、手部肿胀和抗U1 RNP抗体水平升高而被诊断为患有MCTD。在诊断为MCTD一年后，她因出现呼吸困难和胫前水肿而入住我院。通过超声心动图和导管检查确诊为肺动脉高压。在使用泼尼松龙和依那普利（10毫克/天）治疗的三个月期间，肺动脉压力和肺动脉阻力下降并逐渐恢复正常。肺动脉高压是MCTD的胎儿并发症之一，据报道5年死亡率低于50%。然而，尚未确立针对MCTD合并肺动脉高压的成功治疗方法。我们认为ACE抑制剂可能有助于控制MCTD合并的肺动脉高压，并可改善MCTD的预后。
