Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population.
Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population.
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DOI：
10.3899/jrheum.101243
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发表时间：
2011-10
期刊：
影响因子：
--
通讯作者：
PHAROS Investigators
中科院分区：
文献类型：
--
作者：
Hinchcliff M;Fischer A;Schiopu E;Steen VD;PHAROS Investigators
肺动脉高压(PAH)增加了系统性硬化症(SSC)的死亡率。多中心PHAROS(肺高压评估和对硬皮病预后的认识)登记前瞻性地跟踪了SSC的高危人群或合并有肺动脉高压(PH)的患者。在这里，我们描述了自研究开始以来的前18个月内登记的受试者的注册设计和基线特征。符合以下条件的高危受试者可被归类为肺动脉高压前期患者：1)一氧化碳弥散量(DLCO)和一氧化碳弥散量(DLCO)预测为55%，2)预计用力肺活量/DLCO比值≥为1.6，或3)超声心动图估计右室收缩压为35毫米汞柱。右心导管术确诊的肺动脉高压(前6个月内平均肺动脉压≥为25 mm Hg)分为肺动脉高压、左心疾病继发的肺静脉高压和间质性肺疾病所致的肺高压。收集基线和半年一次的人口统计、临床和实验室数据以及患者报告的健康问卷。PHAROS有237个科目注册。大多数是高加索人(73%)和女性(87%)。其中PAH前期166例，明确PH 71例(PAH 49例，PVH 7例，PH-ILD 15例)。Pharos是美国和加拿大最大的SSC受试者队列，这些受试者是PAH事件的高危人群或与之相关的高危人群。针对PAH的特异性治疗被批准用于49/71名RHC确诊的PAH患者。对PHAROS登记数据的分析将有助于确定SSc患者发生PAH的危险因素，并增强我们对SSc-PAH过程的了解。
Pulmonary arterial hypertension (PAH) increases mortality in systemic sclerosis (SSc). The multicenter PHAROS (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) registry prospectively follows SSc subjects at high-risk for, or with incident pulmonary hypertension (PH). Herein, we describe registry design and baseline characteristics of subjects enrolled during the first 18 months since study inception. High-risk subjects are enrolled and classified as Pre-PAH if they have 1) carbon monoxide diffusing capacity (DLCO) < 55% predicted, 2) % predicted forced vital capacity/DLCO ratio ≥1.6, or 3) an estimated right ventricular systolic pressure > 35 mm Hg on echocardiography. Subjects with right heart catheterization confirmed incident PH (mean pulmonary artery pressure ≥ 25 mm Hg within previous 6 months) are subclassified into PAH, pulmonary venous hypertension secondary to left-sided heart disease (PVH), and PH due to interstitial lung disease (PH-ILD). Baseline and biannual demographic, clinical, and laboratory data as well as patient-reported health questionnaires are collected. There are 237 subjects enrolled in PHAROS. The majority are Caucasian (73%) and women (87%). There are 166 Pre-PAH and 71 Definite PH subjects (49 PAH, 7 PVH, and 15 PH-ILD). PHAROS is the largest U.S. and Canadian cohort of SSc subjects at high-risk for or with incident PAH. PAH-specific therapies are approved for 49/71 subjects with RHC-confirmed-PAH. Analyses of PHAROS registry data will permit identification of risk factors for the development of PAH amongst SSc patients at high-risk for PAH and enhance our understanding of the course of SSc-PAH.
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影响因子：
158.5
作者：
Galiè, N;Ghofrani, HA;Simonneau, G
通讯作者：
Simonneau, G
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5.5
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168.9
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