Furst Daniel E
Geffen School of Medicine at the University of California in Los Angeles, 1000 Veteran Avenue Room 32-59, Los Angeles, CA 90095, USA.
Ann N Y Acad Sci. 2007 Jun;1107:410-6. doi: 10.1196/annals.1381.043.
Pulmonary arterial hypertension (PAH) has poor prognosis in systemic sclerosis (SSc), and at present the "gold standard" for diagnosis and follow-up of PAH in SSc is right heart catheterization (RHC) but it would be very useful to have a noninvasive way to follow these patients. Using the OMERACT criteria for validation of measures of response, the only fully validated measure for PAH in SSc has been the 6-min walking test. Multiple other measures are particularly valid (e.g., echocardiography, brain natriuretic protein [BNP], FV/DLCO) while a few are unlikely ever to be validated for various reasons (e.g., symptoms, MRA). A Delphi exercise among 78 experts (EPOSS) has been done and has developed a consensus document consisting of eight domains (lung vascular/PAP, exercise capacity, cardiac function, dyspnea, discontinuation of treatment, quality of life, lung parenchymal, and global state), which can be used and must be tested.
肺动脉高压（PAH）在系统性硬化症（SSc）中的预后较差，目前SSc中PAH诊断和随访的“金标准”是右心导管检查（RHC），但拥有一种非侵入性方法来跟踪这些患者将非常有用。使用OMERACT标准来验证反应测量指标，SSc中PAH唯一经过充分验证的测量指标是6分钟步行试验。其他多种测量指标也特别有效（例如，超声心动图、脑钠肽[BNP]、FV/DLCO），而一些指标由于各种原因不太可能得到验证（例如，症状、磁共振血管造影[MRA]）。已经在78位专家中进行了德尔菲调查（EPOSS），并制定了一份由八个领域（肺血管/肺动脉压、运动能力、心功能、呼吸困难、治疗中断、生活质量、肺实质和整体状态）组成的共识文件，该文件可以使用且必须进行测试。
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