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首页> 外文期刊>Nature clinical practice. Rheumatology >Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis.
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Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis.

机译：系统性硬化症患者的肺动脉高压的诊断。

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BACKGROUND: A 42-year-old woman with limited cutaneous systemic sclerosis presented with rapid-onset dyspnea on exertion, which had developed over the previous 8 weeks. She had not experienced any dyspnea before this period. Transthoracic Doppler echocardiography performed 6 months before presentation demonstrated an estimated right ventricular systolic pressure of 32 mmHg. Lung function tests also performed at that time revealed a decreased diffusion capacity for carbon monoxide of 54% and normal lung volumes, and high-resolution CT scan of the lungs was normal. INVESTIGATIONS: Physical investigation, CBC, analysis of C-reactive protein and pro-brain natriuretic peptide, transthoracic Doppler echocardiography, six-minute walk test, lung function tests including diffusion capacity for carbon monoxide, right heart catheter, high-resolution CT scan, and ventilation/perfusion scan. DIAGNOSIS: Pulmonary arterial hypertension associated with limited cutaneous systemic sclerosis. MANAGEMENT: Treatment with oral anticoagulation therapy and the endothelin-receptor antagonist bosentan. Monitoring of adverse effects of bosentan therapy was performed using liver function tests.
机译：背景：一名42岁的皮肤全身性硬化症患者，在运动时表现为快速发作的呼吸困难，在过去的8周中已经发展。在此之前，她没有呼吸困难。在就诊前6个月进行的经胸多普勒超声心动图检查显示右心室收缩压估计为32 mmHg。当时还进行了肺功能测试，发现一氧化碳的扩散能力降低了54％，肺容积正常，肺的高分辨率CT扫描正常。调查：体格检查，CBC，C反应蛋白和脑钠肽前体的分析，经胸多普勒超声心动图，六分钟步行测试，肺功能测试，包括一氧化碳的扩散能力，右心导管，高分辨率CT扫描，和通气/灌注扫描。诊断：肺动脉高压伴有限的皮肤全身性硬化症。管理：口服抗凝治疗和内皮素受体拮抗剂波生坦治疗。使用肝功能测试监测波生坦疗法的不良反应。

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