Measuring outcomes in PAH - The gap between the measures that are used and their validity

被引：2

作者：

Furst, Daniel E. ^{[1
]}

机构：

[1] Univ Calif Los Angeles, Geffen Sch Med, Los Angeles, CA 90095 USA

来源：

AUTOIMMUNITY, PT C: THE MOSAIC OF AUTOIMMUNITY | 2007年 / 1107卷

关键词：

pulmonary arterial hypertension; systemic sclerosis; outcome measurements;

D O I：

10.1196/annals.1381.043

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页码：410 / 416

页数：7

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